Blood & Blood Disorders - Complete MSN Topic, Nursing notes

🩸 Medical Surgical Nursing – NORCET 2025

Blood &
Blood Disorders

Complete MSN notes on Blood composition, all Anemias, Leukemia, Lymphoma, Multiple Myeloma, Hemophilia, ITP, DIC, Polycythemia, Blood groups, Transfusion reactions — with mnemonics, nursing care and high-yield MCQs for NORCET, AIIMS & State Nursing Exams 2025.

🩸 Blood Composition

💉 All Anemias

🦠 Leukemia & Lymphoma

🩺 Bleeding Disorders

🏥 Transfusion Reactions

❓ 18 MCQs

🩸

Blood – Composition & Normal Values

Foundation knowledge — tested directly in NORCET

Blood is a specialized connective tissue and body fluid that circulates through the cardiovascular system. It constitutes approximately 8% of total body weight (≈5 litres in adults). Blood has two main components: Plasma (55%) and Formed elements (45%) — red blood cells (RBCs), white blood cells (WBCs), and platelets.

🔴

RBC (Erythrocytes)

M: 4.5–5.5 M/µL
F: 3.8–4.8 M/µL

Biconcave disc | No nucleus | Life span 120 days | Contain Haemoglobin | Destroyed in spleen (graveyard of RBC)

⚪

WBC (Leukocytes)

4,000–11,000/µL

Immune defence | 5 types: N,E,B,L,M | Life span: hours–years | Produced in bone marrow (most) & lymph nodes

🟡

Platelets (Thrombocytes)

1.5–4 Lakh/µL (150,000–400,000)

Smallest formed element | No nucleus | Life span 8–10 days | Primary haemostasis | Made from megakaryocytes

🫀

Haemoglobin (Hgb)

M: 13.5–17.5 g/dL
F: 12–15.5 g/dL

Iron-containing protein | Carries O₂ and CO₂ | HbA = normal adult | HbF = fetal (higher O₂ affinity)

💛

Plasma

55% of blood volume

90% water | Contains proteins (albumin, globulin, fibrinogen) | Carries nutrients, hormones, waste products

🔵

Haematocrit (PCV)

M: 40–54%
F: 36–48%

% of blood volume occupied by RBCs | Rule of 3: Hgb × 3 ≈ Haematocrit | Low PCV = anaemia

🔬 WBC Differential Count – Normal Values

WBC Type	Normal %	Function	Mnemonic
Neutrophils	50–70%	First line defence; phagocytosis of bacteria	Never
Eosinophils	1–4%	Allergic reactions, parasitic infections	Let
Basophils	0–1%	Allergic reactions; releases histamine & heparin	Monkeys
Lymphocytes	20–40%	Immune response (B & T cells)	Eat
Monocytes	2–8%	Phagocytosis; become macrophages in tissue	Bananas

🧠

Mnemonic – WBC Differential (High to Low %): "Never Let Monkeys Eat Bananas"

Neutrophils – 50–70%

Lymphocytes – 20–40%

Monocytes – 2–8%

Eosinophils – 1–4%

Basophils – 0–1%

🅰️ Blood Groups – ABO & Rh System

Blood Group	Antigen on RBC	Antibody in Plasma	Can Donate To	Can Receive From
A	A antigen	Anti-B	A, AB	A, O
B	B antigen	Anti-A	B, AB	B, O
AB	A & B antigens	None	AB only	A, B, AB, O (Universal Recipient)
O	None	Anti-A & Anti-B	A, B, AB, O (Universal Donor)	O only

🧠

Key Blood Group Rules

O Negative = Universal DONOR (safe for all patients in emergency)

AB Positive = Universal RECIPIENT (can receive any blood)

Rh+ = Has Rh(D) antigen on RBC surface

Rh− = Does NOT have Rh(D) antigen

Erythroblastosis Fetalis = Rh− mother + Rh+ baby → Anti-D antibodies attack fetal RBCs

👉 Erythroblastosis Fetalis Prevention = Anti-D immunoglobulin (Rho-GAM) given to Rh− mother at 28 weeks + within 72 hrs of delivery

🏭 Haemopoiesis (Blood Cell Formation)

Site	Age / Stage	Cells Produced
Yolk Sac	0–2 months fetal life	Primitive RBCs (megaloblastic)
Liver & Spleen	2–7 months fetal life	RBCs, WBCs, Platelets
Bone Marrow	From 5th fetal month → throughout life	All blood cells (RBC, WBC, Platelets)
Lymph Nodes & Thymus	Throughout life	Lymphocytes (T cells from thymus, B cells from lymph nodes/bone marrow)

💚

Erythropoietin (EPO): Hormone produced by the kidneys (90%) in response to hypoxia → stimulates RBC production in bone marrow. In chronic kidney disease → ↓EPO → anaemia (anaemia of chronic renal failure). Treatment = recombinant EPO (Darbepoetin).

💉

Anaemia – Classification & All Types

Most common blood disorder — know all types, causes, CBC changes and treatment

Anaemia is defined as a reduction in RBC mass, haemoglobin concentration, or haematocrit below normal values, resulting in decreased oxygen-carrying capacity. WHO defines anaemia as: Hgb <13 g/dL in men | Hgb <12 g/dL in women | Hgb <11 g/dL in pregnant women.

🧠

Mnemonic – Types of Anaemia by Cause: "DEAD HEM"

Deficiency (Iron, B12, Folate)

Endocrine/Renal (EPO deficiency)

Aplastic (bone marrow failure)

Dilutional (IV fluids, pregnancy)

Haemolytic (RBC destruction)

Erythrocyte defects (Sickle cell, Thalassemia)

Malignancy (chronic disease)

📊 Anaemia Classification by MCV (CBC)

Type by MCV	MCV	Causes	Blood Film
Microcytic (small RBCs)	<80 fL	Iron deficiency anaemia, Thalassemia, Sideroblastic anaemia, Anaemia of chronic disease	Small, pale (hypochromic) RBCs
Normocytic (normal RBCs)	80–100 fL	Acute blood loss, Haemolytic anaemia, Aplastic anaemia, Renal failure, Mixed deficiency	Normal-sized RBCs, reduced number
Macrocytic (large RBCs)	>100 fL	Vitamin B12 deficiency, Folate deficiency, Hypothyroidism, Liver disease, Alcohol	Large oval RBCs (macro-ovalocytes) + hypersegmented neutrophils

🧠

Mnemonic – MCV Types: "MiNo Ma"

Microcytic = Iron + Thalassemia + Sideroblastic + Chronic disease

Normocytic = Acute loss + Haemolytic + Aplastic + Renal

Macrocytic = Megaloblastic (B12/Folate) + Liver + Alcohol + Hypothyroid

🔴

Iron Deficiency Anaemia (IDA)

Most Common Anaemia Worldwide

⭐ Most Common Anaemia

Type	Microcytic Hypochromic
Causes	Poor intake, Malabsorption, Chronic blood loss (menorrhagia, GI bleed), Pregnancy
CBC	↓Hgb, ↓MCV (<80), ↓MCHC, ↑RDW, ↑TIBC, ↓Serum Ferritin (earliest marker)
Clinical features	Fatigue, pallor, koilonychia (spoon nails), angular stomatitis, glossitis, PICA (craving non-food items)
Specific Signs	Koilonychia, Angular stomatitis, Plummer-Vinson syndrome (dysphagia + IDA + web)
Treatment	Oral ferrous sulphate (elemental iron 200mg/day) for 3–6 months; IV iron if oral not tolerated
Dietary sources	Red meat, liver, leafy greens; Vitamin C enhances absorption; Tea/coffee inhibits

🔴 Serum ferritin = earliest lab marker of IDA. Koilonychia + PICA = classic signs. Reticulocyte count rises 5–7 days after iron therapy starts — confirms response!

🔵

Megaloblastic Anaemia

B12 & Folate Deficiency

Macrocytic Anaemia

Type	Macrocytic (Megaloblastic)
Vitamin B12 sources	Animal products only (meat, fish, eggs, dairy) — strict vegetarians at risk
Folate sources	Green leafy vegetables, fruits, liver — destroyed by cooking
CBC	↑MCV (>100), Macro-ovalocytes, Hypersegmented neutrophils (>5 lobes), ↓reticulocytes
B12 deficit extra	Neurological: subacute combined degeneration of spinal cord (posterior + lateral columns)
Folate deficit extra	Neural tube defects in fetus (spina bifida) — folic acid supplements in pregnancy!
Treatment	B12: Cyanocobalamin IM monthly (if pernicious/malabsorption). Folate: Folic acid 5mg/day oral

🔵 Hypersegmented neutrophils (>5 lobes) on blood film = HALLMARK of megaloblastic anaemia. B12 deficiency causes neurological damage (subacute combined degeneration); Folate deficiency does NOT cause neurological damage!

💜

Pernicious Anaemia

Autoimmune B12 deficiency

Autoimmune – Intrinsic Factor Absent

Cause	Autoimmune destruction of gastric parietal cells → no Intrinsic Factor (IF) → B12 cannot be absorbed in terminal ileum
Key feature	Anti-IF antibodies and Anti-parietal cell antibodies present
Achlorhydria	No hydrochloric acid (no parietal cells) → Schilling test confirms
Neurological	Subacute Combined Degeneration (SCD) — posterior column (vibration/proprioception) + lateral column (UMN signs)
Schilling Test	Radioactive B12 oral + IM B12 → if urinary B12 low = malabsorption confirmed
Treatment	IM Cyanocobalamin (Vit B12) — monthly injections LIFELONG (cannot absorb oral B12)

💜 Pernicious anaemia = LIFELONG IM B12 injections (cannot use oral as absorption requires IF which is absent). Schilling test is used for diagnosis. Associated with other autoimmune diseases (thyroid, Type 1 DM).

🟠

Aplastic Anaemia

Bone Marrow Failure

Pancytopenia

Definition	Failure of bone marrow to produce adequate blood cells → PANCYTOPENIA (↓RBC + ↓WBC + ↓Platelets)
Causes	Idiopathic (most common), Drugs (chloramphenicol, benzene, cytotoxics), Radiation, Viral (hepatitis, Parvovirus B19), Autoimmune
CBC	Pancytopenia: ↓Hgb + ↓WBC + ↓Platelets | Normocytic RBCs | ↓Reticulocyte count
Gold standard diagnosis	Bone Marrow Biopsy = hypocellular/fatty marrow (replacement of marrow by fat cells)
Symptoms	Anaemia symptoms + infections (↓WBC) + bleeding (↓Platelets) simultaneously
Treatment	Mild-moderate: Cyclosporine + Anti-thymocyte globulin (ATG). Severe: Bone Marrow Transplant (BMT) = curative

🟠 Bone Marrow Transplant = ONLY CURE for severe aplastic anaemia. Bone marrow biopsy = "dry tap" or hypocellular marrow = diagnostic hallmark. Chloramphenicol is the most notorious drug causing aplastic anaemia.

🔄

Haemolytic Anaemia

Excessive RBC Destruction

Premature RBC destruction

Definition	RBC life span reduced below 120 days → accelerated destruction → anaemia + hyperbilirubinaemia + reticulocytosis
Intrinsic causes	Sickle cell disease, Thalassemia, G6PD deficiency, Hereditary spherocytosis
Extrinsic causes	Autoimmune (AIHA), Mechanical (prosthetic valves), Infections (malaria), Drugs, Transfusion reactions
Lab findings	↑Indirect bilirubin, ↑LDH, ↓Haptoglobin, ↑Reticulocyte count (bone marrow response)
Coombs test	Direct Coombs (DAT): positive in autoimmune haemolytic anaemia
Site of destruction	Intravascular (within blood vessels) or Extravascular (spleen/liver — most common)

🔄 ↑Reticulocyte count + ↑Indirect bilirubin + ↓Haptoglobin = HAEMOLYSIS triad. Direct Coombs test (+) = autoimmune haemolytic anaemia. Spleen = main site of RBC destruction in most haemolytic anaemias.

🌙

Sickle Cell Anaemia (SCA)

Haemoglobinopathy – HbS

Autosomal Recessive

Mutation	Point mutation: Glutamic acid → Valine at position 6 of β-globin chain → HbS formation
Triggers for sickling	Hypoxia, Dehydration, Acidosis, Cold, Infection, High altitude, Stress
Clinical features	Vaso-occlusive crisis (pain), Acute Chest Syndrome, Stroke, Splenic sequestration, Dactylitis (hand-foot syndrome in infants), Priapism
Diagnosis	Hgb electrophoresis (HbSS), Peripheral smear (sickle cells + target cells)
Spleen	Auto-splenectomy by age 5 → susceptible to encapsulated bacteria (Pneumococcus, H. influenzae)
Treatment	Hydroxyurea (↑HbF — prevents sickling); Folic acid; Analgesics; Hydration; O₂; Blood transfusion in crisis
Curative	Bone Marrow Transplant (BMT) — only cure

🌙 Hydroxyurea = drug of choice for SCA (increases HbF levels). Auto-splenectomy → prophylactic penicillin V + pneumococcal vaccine needed. Dactylitis = first manifestation in infants (painful swelling of hands/feet).

🧬

Thalassemia

Alpha & Beta Thalassemia

Genetic Haemoglobinopathy

Beta Thalassemia Major	No β-globin chains → severe anaemia from 3–6 months (when HbF → HbA switch occurs)
Clinical features	Chipmunk facies (frontal bossing, prominent cheekbones), Hepatosplenomegaly, Growth retardation, Iron overload
Skull X-ray	"Hair on end" appearance (diploë expansion due to extramedullary haematopoiesis)
Diagnosis	Hgb electrophoresis: ↑HbF, ↑HbA2, absent/reduced HbA; Target cells on smear
Treatment	Regular blood transfusions (every 3–4 weeks) + Desferrioxamine (iron chelation — prevents iron overload)
Curative	BMT (bone marrow transplant)
Beta Thal Minor	Trait — asymptomatic or mild anaemia; important for genetic counselling

🧬 "Hair on end" skull X-ray = Thalassemia major (very high-yield). Desferrioxamine/Deferasirox = Iron chelation therapy for transfusion-related iron overload (haemochromatosis). Chipmunk facies = classic facies.

💊

G6PD Deficiency

Glucose-6-Phosphate Dehydrogenase Deficiency

X-linked Recessive

Inheritance	X-linked recessive — mainly affects males
Mechanism	Lack of G6PD → RBCs cannot handle oxidative stress → Heinz body formation → haemolysis
Triggers	Primaquine, Dapsone, Fava beans (broad beans), Infections, Naphthalene (moth balls)
Blood film	Heinz bodies (denatured Hgb inclusions), Bite cells
Management	Avoid triggers; Supportive care; Blood transfusion if severe

💊 G6PD deficiency is the MOST COMMON enzymopathy worldwide. "Fava beans + haemolysis = G6PD deficiency." Heinz bodies = denatured Hgb — seen on special stain (not standard Giemsa).

📈 Polycythemia (Erythrocytosis)

Polycythemia = abnormal increase in RBC mass / Hgb / Haematocrit. The most important primary form is Polycythemia Vera (PV) — a myeloproliferative neoplasm.

Feature	Polycythemia Vera (Primary)	Secondary Polycythemia
Cause	JAK2 V617F mutation (present in 97%) — autonomous RBC overproduction	Response to ↑EPO: High altitude, COPD, Renal tumour, Smoking
EPO level	↓ Decreased (suppressed)	↑ Increased
CBC	↑RBC + ↑WBC + ↑Platelets (all three lines elevated)	Only ↑RBC
Symptoms	Plethoric (ruddy) face, Pruritus after warm bath (aquagenic pruritus), Headache, Hypertension, Splenomegaly, Risk of thrombosis	Symptoms of hypoxia + polycythemia
Aquagenic pruritus	Itching after warm shower/bath — PATHOGNOMONIC of PV	Absent
Treatment	Phlebotomy (venesection) — first line! Hydroxyurea, Aspirin (antiplatelet)	Treat underlying cause; supplemental O₂

🚨

High-Yield! Aquagenic pruritus (itching after warm bath) = PATHOGNOMONIC of Polycythemia Vera. JAK2 mutation present in 97% = molecular marker. Phlebotomy = first-line treatment. PV can transform to AML or myelofibrosis.

🦠

Malignant Blood Disorders

Leukemia, Lymphoma & Multiple Myeloma — high-yield for NORCET

🦠 Leukemia – Classification & Key Features

Leukemia is a malignant clonal proliferation of leukocytes (or their precursors) in the bone marrow. Classified as Acute (rapid, blasts >20%) or Chronic (slower, mature cells), and by cell type: Lymphoid or Myeloid.

🧠

Mnemonic – Leukemia Types & Age Groups: "ALL Children, AML Adults, CLL Old, CML Middle"

ALL = Most common in Children (peak 2–5 yrs) — most common childhood cancer

AML = Most common acute leukemia in Adults

CLL = Most common leukemia in Western Elderly (>60 yrs)

CML = Middle age adults (40–60 yrs); Philadelphia chromosome

👦

ALL – Acute Lymphoblastic Leukemia

Most common childhood cancer

Children peak 2–5 years

Cell origin	Lymphoid precursors (B-cell most common; T-cell in older children/adults)
Key marker	TdT (Terminal deoxynucleotidyl Transferase) positive — marker of lymphoid blasts
Good prognosis	Age 1–9 years, Low WBC, Hyperdiploidy, TEL-AML1 translocation
CNS involvement	Common — headache, cranial nerve palsies, papilloedema; CSF analysis needed
Treatment	Induction: Vincristine + Prednisolone + Asparaginase | Consolidation + Maintenance (2–3 years total) | CNS prophylaxis (intrathecal methotrexate)
Prognosis	Best of all leukemias: 90% remission in children

👦 TdT positive = hallmark of ALL. Best prognosis of all leukemias in children (90% cure rate). CNS prophylaxis with intrathecal methotrexate is essential to prevent CNS relapse.

🔴

AML – Acute Myeloid Leukemia

Most common acute leukaemia in adults

Adults (peak 65 yrs)

Cell origin	Myeloid precursors (granulocytic, monocytic, erythroid, megakaryocytic)
Auer rods	Pink needle-like inclusions in blast cytoplasm — PATHOGNOMONIC of AML (especially AML-M3)
APL (AML-M3)	Acute Promyelocytic Leukemia: t(15;17) translocation → PML-RARα fusion → risk of DIC; treat with ATRA (all-trans retinoic acid)
DIC risk	APL (AML-M3) → severe DIC — most dangerous subtype
Treatment	7+3 regimen: Cytarabine × 7 days + Anthracycline × 3 days | APL: ATRA + Arsenic trioxide
Prognosis	Worse than ALL; 40–50% CR in adults; BMT considered

🔴 Auer rods = PATHOGNOMONIC of AML. APL (M3) = t(15;17) + DIC risk + treated with ATRA. DIC + leukemia in exams = always think APL (AML-M3)!

👴

CLL – Chronic Lymphocytic Leukemia

Most common Western leukaemia

Elderly (>60 years)

Cell origin	Mature B lymphocytes (non-functional)
Key feature	Smudge cells (Basket cells) on blood film — fragile lymphocytes that smear during preparation
CBC	Markedly elevated lymphocyte count (>5,000/µL); lymphocytes look mature but non-functional
Hypogammaglobulinaemia	Recurrent bacterial infections due to ↓immunoglobulins
Autoimmune	AIHA and ITP can occur (Coombs positive haemolysis)
Treatment	Watch and wait (asymptomatic early stage); Fludarabine + Cyclophosphamide + Rituximab (FCR); Ibrutinib (BTK inhibitor)

👴 Smudge/Basket cells = HALLMARK of CLL on blood film. Most indolent leukemia — "watch and wait" in early stages. Transformation to aggressive lymphoma = Richter's transformation.

🧬

CML – Chronic Myeloid Leukemia

Philadelphia Chromosome

Philadelphia Chromosome t(9;22)

Cell origin	Myeloid stem cell — all granulocyte stages present
Philadelphia chromosome	t(9;22) translocation → BCR-ABL fusion gene → constitutive tyrosine kinase activity → uncontrolled proliferation
Tri-phase disease	Chronic phase (stable, 3–5 yrs) → Accelerated phase → Blast crisis (like acute leukemia)
Blood film	All stages of granulocytes (full myeloid spectrum): myeloblasts → promyelocytes → myelocytes → neutrophils
Splenomegaly	Massive splenomegaly — most prominent finding
Treatment	Imatinib (Gleevec) = tyrosine kinase inhibitor (TKI) — REVOLUTIONISED CML treatment; highly effective

🧬 Philadelphia chromosome [t(9;22)] = BCR-ABL = CML. Imatinib (Gleevec) = first targeted cancer therapy — Nobel Prize associated! "All generations" of granulocytes on smear = CML. Low LAP score (Leukocyte Alkaline Phosphatase) in CML.

🏥 Lymphoma – Hodgkin's & Non-Hodgkin's

Feature	Hodgkin's Lymphoma (HL)	Non-Hodgkin's Lymphoma (NHL)
Hallmark Cell	Reed-Sternberg Cell ("Owl-eye" appearance) — PATHOGNOMONIC	No Reed-Sternberg cells; malignant B or T cells
Age	Bimodal: 15–35 yrs & >55 yrs	Older adults; any age
Spread	Contiguous (orderly, node-to-node)	Non-contiguous (random, widespread)
Presentation	Painless cervical lymphadenopathy, B symptoms (fever, night sweats, weight loss >10%)	Widespread lymphadenopathy, extranodal involvement more common
B Symptoms	Fever + Night Sweats + Weight loss >10% in 6 months (poor prognosis marker)	Same B symptoms can occur
Pel-Ebstein Fever	Cyclical fever (days of high temp + days of normal) — classic in HL	Less characteristic
Prognosis	Better prognosis overall (curable ~80%)	Variable — depends on subtype
Treatment	ABVD regimen: Adriamycin + Bleomycin + Vinblastine + Dacarbazine + Radiotherapy	CHOP ± Rituximab (R-CHOP); depends on subtype

🚨

MOST High-Yield! Reed-Sternberg (RS) cell = "Owl Eye" cell = PATHOGNOMONIC of Hodgkin's Lymphoma. B symptoms = Fever + Night sweats + Weight loss >10%. Pel-Ebstein fever = cyclical fever pattern in HL. ABVD = standard chemotherapy for HL.

🦴 Multiple Myeloma (MM)

Multiple Myeloma is a malignant proliferation of plasma cells in the bone marrow producing monoclonal immunoglobulin (M-protein / paraprotein). It is the second most common haematological malignancy (after CLL in Western countries).

🧠

Mnemonic – CRAB Criteria for Multiple Myeloma

C = Calcium ↑ (Hypercalcaemia — bone destruction releases Ca²⁺)

R = Renal failure (Bence-Jones proteins clog tubules)

A = Anaemia (bone marrow replaced by plasma cells)

B = Bone lesions (lytic "punched-out" lesions on X-ray; pathological fractures)

Additional features: Rouleaux formation (RBCs stack like coins on smear), Bence-Jones protein in urine, M-spike on SPEP, Recurrent infections

Feature	Details
Diagnosis	Bone marrow biopsy (>10% plasma cells) + Serum/Urine protein electrophoresis (M-spike) + Skeletal survey (lytic lesions)
Bence-Jones Protein	Free light chains (kappa or lambda) in urine — classic marker of MM; can cause renal failure
Rouleaux formation	RBCs stack like coins on peripheral blood smear — due to high paraprotein levels
Bone lesions	"Punched-out" lytic lesions on skull X-ray (rain-drop skull); Bone pain (back/ribs most common); Pathological fractures
Treatment	Bortezomib (proteasome inhibitor) + Thalidomide/Lenalidomide + Dexamethasone (VTD regimen); Autologous stem cell transplant
Prognosis	Median survival 5–7 years; incurable but manageable

🩺

Bleeding Disorders

Hemophilia, ITP, DIC, Von Willebrand Disease — critical nursing topics

💧

Hemophilia A

Factor VIII Deficiency

Most Common Hemophilia – X-linked Recessive

Deficiency	Factor VIII (anti-haemophilic factor)
Inheritance	X-linked recessive — affects males; females are carriers
Severity	Mild (>5%), Moderate (1–5%), Severe (<1%) Factor VIII levels
Bleeding pattern	Haemarthrosis (joint bleeds — most common), Muscle bleeds, Deep tissue, ICH
Lab findings	↑aPTT | Normal PT | Normal Platelet count | Normal Bleeding time
Treatment	Factor VIII concentrate (recombinant or plasma-derived); DDAVP (Desmopressin) for mild cases; avoid Aspirin/NSAIDs

💧 Haemarthrosis = most common site of bleeding in haemophilia. Target joint = repeatedly affected joint (knee most common). aPTT prolonged; PT normal = Factor VIII/IX/XI deficiency (intrinsic pathway). DDAVP releases stored Factor VIII from endothelium.

💙

Hemophilia B (Christmas Disease)

Factor IX Deficiency

X-linked Recessive – Factor IX

Deficiency	Factor IX (Christmas factor)
Inheritance	X-linked recessive — same as Hemophilia A
Clinical features	Identical to Hemophilia A (haemarthrosis, muscle bleeds)
Lab findings	↑aPTT | Normal PT | Normal Platelet count (same as Hem A)
Differentiation from A	Specific Factor assay: Factor VIII normal (Hem B); Factor IX low (Hem B)
Treatment	Factor IX concentrate; No DDAVP effect (unlike Hem A)

💙 Hemophilia B = "Christmas Disease" (named after Stephen Christmas, first patient described). Same lab pattern as A — differentiated only by specific factor assay. 4× less common than Hemophilia A.

🟣

ITP – Immune Thrombocytopenic Purpura

Autoimmune platelet destruction

Autoimmune – Anti-platelet IgG

Definition	Autoimmune destruction of platelets — IgG antibodies (anti-GpIIb/IIIa) coat platelets → destroyed in spleen
Types	Acute ITP: Children after viral infection (self-limiting, resolves in 6 months). Chronic ITP: Adults, especially young women
Lab findings	↓Platelet count (<100,000; often <20,000) | Normal PT + aPTT | Normal Hgb + WBC | ↑Megakaryocytes in bone marrow
Clinical features	Petechiae, Purpura, Ecchymosis, Mucosal bleeding, Menorrhagia; No splenomegaly (unlike hypersplenism)
Serious risk	Intracranial haemorrhage (platelet <10,000)
Treatment	Prednisolone (first line); IVIG (rapid increase in platelets); Anti-D immunoglobulin; Splenectomy (refractory); Romiplostim (TPO agonist)

🟣 Key: Isolated thrombocytopenia + Normal PT/aPTT = ITP. Bone marrow shows ↑megakaryocytes (platelets being made but destroyed). No splenomegaly in ITP! Platelet transfusion is INEFFECTIVE (destroyed immediately). Splenectomy = definitive for refractory ITP.

🔥

DIC – Disseminated Intravascular Coagulation

Life-threatening coagulopathy

EMERGENCY – Consumption Coagulopathy

Definition	Widespread abnormal activation of coagulation system → simultaneous thrombosis AND bleeding → consumption of all clotting factors and platelets
Causes	Sepsis (most common), Obstetric complications (HELLP, abruption, retained placenta), Trauma, Burns, Malignancy (APL-M3)
Lab findings	↑PT + ↑aPTT + ↑INR | ↓Fibrinogen | ↑D-dimer (most sensitive) | ↓Platelets | Microangiopathic haemolytic anaemia (schistocytes)
D-dimer	Most sensitive marker of DIC — fibrin degradation product
Clinical features	Bleeding from multiple sites (IV sites, gums, urine, stool) + Organ failure + Thrombosis
Treatment	TREAT THE CAUSE first! FFP (Fresh Frozen Plasma) — replaces all clotting factors; Platelet transfusion if <50,000; Cryoprecipitate (fibrinogen); Heparin (controversial — thrombotic phase)

🔥 D-dimer = most sensitive test for DIC. ↑PT + ↑aPTT + ↓Fibrinogen + ↓Platelets + ↑D-dimer = DIC diagnosis. FFP contains ALL clotting factors. TREAT THE CAUSE is the most important intervention! Sepsis = most common cause.

🩺

Von Willebrand Disease (VWD)

Most Common Inherited Bleeding Disorder

Autosomal Dominant – VWF Deficiency

Deficiency	Von Willebrand Factor (VWF) — needed for platelet adhesion AND carries Factor VIII
Inheritance	Autosomal dominant (most types) — affects both sexes equally
Lab findings	↑Bleeding time | ↑aPTT (mild — VWF carries Factor VIII) | Normal PT | ↓Ristocetin cofactor assay
Clinical features	Mucocutaneous bleeding: epistaxis (most common), Menorrhagia, Easy bruising, Gum bleeding (platelet-type bleeding)
Difference from Hemophilia	VWD = mucocutaneous bleeding + ↑BT; Hemophilia = deep bleeds (haemarthrosis) + normal BT
Treatment	DDAVP (Desmopressin) — releases VWF from endothelium (Type 1 VWD); VWF concentrate; Tranexamic acid

🩺 VWD = most common inherited bleeding disorder worldwide. Ristocetin cofactor assay = specific test for VWD. DDAVP = treatment for Type 1 VWD (mild). Prolonged bleeding time + ↑aPTT = VWD (differentiates from hemophilia which has normal BT).

🔗 Coagulation Cascade – Quick Reference

Pathway	Factors Involved	Measured By	Deficient In
Extrinsic Pathway	Factor III (Tissue Factor) + Factor VII	PT (Prothrombin Time) / INR	Warfarin effect, Liver disease, Vit K deficiency, Factor VII deficiency
Intrinsic Pathway	Factors XII → XI → IX → VIII	aPTT (activated Partial Thromboplastin Time)	Hemophilia A (VIII), Hemophilia B (IX), VWD, Heparin therapy
Common Pathway	Factor X → V → II (Thrombin) → I (Fibrin)	Both PT and aPTT	DIC, Liver failure, Warfarin overdose, Vit K deficiency

🏥

Blood Transfusion & Transfusion Reactions

Nursing responsibilities and reaction management — very high-yield for NORCET

💉 Blood Products – Types & Uses

Product	Contents	Indication	Volume/Unit
Packed RBCs (pRBCs)	RBCs + small amount plasma	Anaemia, Acute blood loss (Hgb <7 or symptomatic)	~250–350 mL; raises Hgb by 1 g/dL per unit
Fresh Frozen Plasma (FFP)	ALL clotting factors + plasma proteins	DIC, Liver disease, Warfarin reversal, Factor deficiencies	~200–250 mL per unit
Platelets	Concentrated platelets	Thrombocytopenia (<10,000), Bleeding with <50,000 platelets	Each unit raises platelets by 5,000–10,000/µL
Cryoprecipitate	Factor VIII, vWF, Fibrinogen, Factor XIII	DIC (low fibrinogen), Hemophilia A, VWD, Massive transfusion	~15–20 mL per unit
Whole Blood	All components	Massive haemorrhage, Exchange transfusion in neonates	450–500 mL per unit
Albumin	Human albumin 4–5% or 20–25%	Hypoalbuminaemia, Shock, Hepatic failure, Burns	Variable

⚠️ Blood Transfusion Reactions – Classification & Management

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MOST IMPORTANT NURSING ACTION in ANY transfusion reaction: STOP THE TRANSFUSION IMMEDIATELY — keep IV line open with normal saline. Notify physician. Send blood and tubing to lab. Monitor vital signs. This applies to ALL types of transfusion reactions — always stop first!

🔴 Acute Haemolytic Reaction

Within minutes–1 hour

• ABO incompatibility — most dangerous
• Fever, chills, back/flank pain (most specific)
• Haemoglobinuria (red/cola urine)
• Hypotension, tachycardia
• DIC, acute renal failure possible
• Most preventable — proper cross-matching

🛑 STOP transfusion | NS infusion | Diuretics | Treat DIC | Notify blood bank

🟠 Febrile Non-Haemolytic Reaction (FNHTR)

During or up to 4 hrs after

• Most common transfusion reaction
• Fever (>1°C rise) + chills
• Due to recipient antibodies against donor WBCs
• No haemolysis — benign
• More common in multitransfused patients

🛑 STOP transfusion | Paracetamol (antipyretic) | Rule out haemolytic reaction first | Leuco-reduced blood prevents future reactions

🔵 Allergic / Urticarial Reaction

During transfusion

• Urticaria (hives), pruritus
• Due to antibodies against plasma proteins
• No fever, no haemolysis
• Usually mild — most common allergic type
• Anaphylaxis if severe (especially IgA-deficient patients)

🛑 STOP transfusion | Antihistamine (Chlorpheniramine) | Mild: may restart slowly after treatment | Anaphylaxis: Epinephrine

🟢 TRALI (Transfusion-Related Acute Lung Injury)

Within 6 hours

• Acute respiratory distress (like ARDS)
• Bilateral pulmonary infiltrates on CXR
• Hypoxia, dyspnoea, fever, hypotension
• Due to donor antibodies against recipient WBCs
• Leading cause of transfusion-related death

🛑 STOP transfusion | Respiratory support / Mechanical ventilation | Supportive care | NO diuretics (not cardiac pulmonary oedema)

💜 Delayed Haemolytic Reaction

3–10 days after transfusion

• Gradual drop in Hgb after initially successful transfusion
• Jaundice, low-grade fever
• Due to anamnestic antibody response (Rh, Kidd, Duffy antigens)
• Direct Coombs test becomes positive
• Usually milder than acute haemolytic

🛑 Monitor Hgb closely | Supportive care | Future transfusions: extended crossmatch needed

🔵 TACO (Transfusion-Associated Circulatory Overload)

During or within 6 hours

• Fluid overload — especially elderly, cardiac/renal patients
• Dyspnoea, hypertension, tachycardia, pulmonary oedema
• JVP raised, bilateral crackles
• Distinction from TRALI: HTN + ↑JVP in TACO; hypotension in TRALI
• Most common in rapid transfusion

🛑 STOP or slow transfusion | Sit upright | Diuretics (Furosemide) | O₂ | Prevent with slower infusion rate

👩‍⚕️ Nursing Care During Blood Transfusion

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Pre-Transfusion Safety Checks

• Verify physician's order — blood product, volume, rate
• Two nurses verify patient ID (name + DOB + hospital number)
• Check blood product label with blood bank form
• Verify ABO and Rh type match
• Check expiry date and inspect for clots/colour change
• Obtain informed consent and baseline vital signs

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Administration Guidelines

• Use blood administration set (170–260 micron filter)
• Start infusion slowly — first 15 minutes at 1 mL/min (watch for reactions)
• pRBCs: complete within 4 hours of leaving blood bank
• Only compatible IV fluid = 0.9% Normal Saline (never dextrose — causes haemolysis!)
• Do NOT add medications to blood bag
• Do NOT warm blood in hot water — use blood warmer if needed

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Monitoring During Transfusion

• Vital signs: Before, 15 min, 30 min, 1 hour, every hour, and after completion
• Stay with patient for first 15 minutes (highest reaction risk)
• Observe for: fever, chills, urticaria, back pain, dyspnoea, haemoglobinuria
• Monitor urine output — minimum 30 mL/hr
• Watch for fluid overload (elderly/cardiac patients)

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Reaction Management (ALL types)

• STOP transfusion IMMEDIATELY — first action always
• Keep IV line open with 0.9% Normal Saline
• Notify physician immediately
• Monitor vital signs continuously
• Return blood bag + tubing to blood bank for analysis
• Obtain blood and urine specimens
• Document everything accurately

👩‍⚕️ General Nursing Care for Blood Disorders

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Anaemia – Nursing Care

• Monitor Hgb, Hct, reticulocyte count; report Hgb <7 g/dL
• Assess for fatigue, pallor, dyspnoea, tachycardia
• Provide supplemental O₂ if SpO₂ <94%
• Energy conservation — rest between activities
• High-iron diet: dark leafy greens, meat, legumes
• Teach about oral iron: take on empty stomach, with Vit C, stools may be dark
• Fall prevention — dizziness/weakness increases fall risk

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Leukemia / Immunosuppression

• Strict infection prevention — reverse isolation if ANC <500
• Hand hygiene — most important infection control measure
• No fresh flowers, fruits with skin, standing water in room
• Daily temperature monitoring — fever in neutropenia = emergency
• Oral care every 2–4 hours — prevent mucositis
• Monitor for signs of sepsis

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Bleeding Disorders / Thrombocytopenia

• Assess for petechiae, ecchymosis, active bleeding sites
• Avoid IM injections if platelet <50,000
• Apply pressure for minimum 5–10 minutes after venipuncture
• Avoid Aspirin, NSAIDs, anticoagulants
• Soft toothbrush; electric razor only
• Platelet <10,000: strict bed rest; no invasive procedures
• ICH prevention: avoid Valsalva, constipation, heavy lifting

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Chemotherapy Care

• Monitor CBC nadir (lowest point) — usually 7–14 days after chemo
• Manage nausea: anti-emetics before and after chemo
• Mucositis care: normal saline + sodium bicarbonate mouth rinses
• Alopecia support and counselling
• Monitor renal function (tumour lysis syndrome risk)
• PICC/central line care — strict aseptic technique
• Hydration: 2–3 L/day to prevent uric acid accumulation

📝 High-Yield MCQs – Blood & Blood Disorders (NORCET 2025)

Q1. The MOST COMMON anaemia worldwide is?

• A) Aplastic Anaemia
• B) Megaloblastic Anaemia
• C) Iron Deficiency Anaemia (IDA)
• D) Haemolytic Anaemia

💡 Tip: IDA = most common anaemia worldwide (WHO). Classic signs: Koilonychia (spoon nails), Angular stomatitis, PICA, Glossitis. Earliest lab marker = ↓Serum Ferritin. CBC: Microcytic hypochromic (↓MCV, ↓MCHC, ↑TIBC, ↑RDW).

Q2. The HALLMARK blood smear finding of Megaloblastic Anaemia is?

• A) Target cells and sickle cells
• B) Auer rods in blast cells
• C) Hypersegmented neutrophils (more than 5 lobes) + macro-ovalocytes
• D) Smudge cells (Basket cells)

💡 Tip: Hypersegmented neutrophils (>5 lobes) = HALLMARK of megaloblastic anaemia (B12 or Folate deficiency). MCV >100 fL. B12 deficiency → neurological damage (SCD of spinal cord). Folate deficiency → neural tube defects in fetus. Folate does NOT cause neurological damage!

Q3. The Universal Blood Donor is?

• A) AB positive
• B) O positive
• C) O negative
• D) AB negative

💡 Tip: O negative = Universal DONOR (no A/B antigens + no Rh antigen = safe for anyone in emergency). AB positive = Universal RECIPIENT (has A + B antigens, so no antibodies against them; Rh positive). O negative is given when no time for crossmatch.

Q4. The MOST IMPORTANT first action when any transfusion reaction is suspected is?

• A) Give antihistamine IV
• B) Slow the infusion rate
• C) STOP the transfusion immediately and keep IV line open with normal saline
• D) Take vital signs and document

💡 Tip: STOP the transfusion = FIRST action for ANY type of transfusion reaction (acute haemolytic, febrile, allergic — all types). Keep IV open with normal saline. NEVER use dextrose (causes RBC haemolysis). Then notify physician + send blood bag to lab.

Q5. Aquagenic pruritus (itching after warm bath) is pathognomonic of?

• A) Iron Deficiency Anaemia
• B) Hodgkin's Lymphoma
• C) Polycythemia Vera
• D) Sickle Cell Disease

💡 Tip: Aquagenic pruritus = PATHOGNOMONIC of Polycythemia Vera (PV). Other features: ruddy face, JAK2 V617F mutation (97%), thrombosis risk, ↑RBC+WBC+PLT. Treatment: Phlebotomy (venesection) = first line. EPO levels ↓ in PV (suppressed).

Q6. The PATHOGNOMONIC cell of Hodgkin's Lymphoma is?

• A) Auer rod in blast cells
• B) Smudge cell (Basket cell)
• C) Reed-Sternberg cell ("Owl-eye" appearance)
• D) Hypersegmented neutrophil

💡 Tip: Reed-Sternberg cell = "Owl eye" giant binucleated cell = PATHOGNOMONIC of Hodgkin's Lymphoma. B symptoms = Fever + Night Sweats + Weight loss >10%. Pel-Ebstein fever = cyclical fever in HL. ABVD chemotherapy = standard treatment.

Q7. The diagnostic hallmark on bone marrow biopsy in Aplastic Anaemia is?

• A) Hypercellular marrow with blasts
• B) Plasma cell infiltration
• C) Hypocellular/fatty marrow replacing haemopoietic tissue
• D) Reed-Sternberg cells in marrow

💡 Tip: Aplastic anaemia = Pancytopenia + Hypocellular marrow (fat replaces blood-forming cells). Gold standard diagnosis = bone marrow biopsy. Most notorious drug cause = Chloramphenicol. BMT = only cure for severe aplastic anaemia.

Q8. The Philadelphia chromosome [t(9;22)] causing BCR-ABL fusion is associated with?

• A) Acute Lymphoblastic Leukemia only
• B) Hodgkin's Lymphoma
• C) Chronic Myeloid Leukemia (CML)
• D) Multiple Myeloma

💡 Tip: Philadelphia chromosome t(9;22) = BCR-ABL = CML. Imatinib (Gleevec) = tyrosine kinase inhibitor = revolutionized CML treatment. CML presents with massive splenomegaly. Can also occur in ALL (poor prognosis). Smear shows all stages of granulocytes.

Q9. A nurse is assessing lab results of a patient with DIC. Which finding is MOST specific for DIC?

• A) Elevated WBC count
• B) Reduced Haemoglobin
• C) Elevated D-dimer with prolonged PT, aPTT and low fibrinogen
• D) Elevated platelet count

💡 Tip: DIC labs = ↑PT + ↑aPTT + ↑D-dimer + ↓Fibrinogen + ↓Platelets + Schistocytes on smear. D-dimer = most sensitive single marker. FFP = first-line treatment (replaces all clotting factors). TREAT THE CAUSE = most important principle. Sepsis = most common cause.

Q10. The most common site of bleeding in Hemophilia A is?

• A) Skin petechiae and purpura
• B) Gum and nasal bleeding
• C) Haemarthrosis (joint bleeding) — knee most common
• D) Intracranial haemorrhage

💡 Tip: Haemarthrosis = most common (hallmark) bleeding in haemophilia. Petechiae/purpura/mucosal = platelet disorders (ITP, VWD). Haemophilia = deep bleeds (joints, muscles). aPTT ↑, PT normal = haemophilia. Factor VIII = Hemophilia A; Factor IX = Hemophilia B (Christmas disease).

Q11. The CRAB criteria for diagnosis of Multiple Myeloma stands for?

• A) Cough, Renal failure, Anaemia, Bone pain
• B) Hypercalcaemia, Renal failure, Anaemia, Bone lesions
• C) Cyanosis, Respiratory failure, Ataxia, Bleeding
• D) Cardiac failure, Renal failure, Alopecia, Bone fractures

💡 Tip: CRAB = Calcium↑ + Renal failure + Anaemia + Bone lesions (lytic, "punched-out," rain-drop skull). Bence-Jones protein in urine = light chains. Rouleaux formation on smear. M-spike on protein electrophoresis. Bortezomib + Thalidomide + Dexamethasone = treatment.

Q12. A child develops fever, chills and haemoglobinuria (red urine) 30 minutes after starting a blood transfusion. The nurse should FIRST?

• A) Slow the transfusion rate
• B) Give paracetamol for fever
• C) Stop the transfusion immediately and keep IV open with normal saline
• D) Check temperature every 15 minutes

💡 Tip: Haemoglobinuria (red/cola urine) + fever + chills + flank pain early in transfusion = ACUTE HAEMOLYTIC REACTION (ABO incompatibility) — most dangerous reaction. FIRST action = STOP transfusion. Haemolysis releases free Hgb → renal failure → DIC risk. Most preventable with proper crossmatch.

Q13. Which is the MOST COMMON leukemia in children?

• A) AML
• B) CML
• C) ALL (Acute Lymphoblastic Leukemia)
• D) CLL

💡 Tip: ALL = most common childhood cancer AND most common childhood leukemia (peak 2–5 years). TdT positive = marker of ALL. 90% CR rate in children. AML = most common acute leukemia in adults. CLL = most common overall in Western elderly. CML = Philadelphia chromosome.

Q14. Sickle Cell Anaemia is caused by a point mutation where?

• A) Glutamine → Valine at position 11 of alpha-chain
• C) Glutamic acid → Valine at position 6 of beta-globin chain
• D) Histidine → Aspartate at position 12 of beta-chain

💡 Tip: SCA mutation: Glu→Val at position 6 of β-globin = HbS. Triggers: hypoxia, dehydration, cold, infection, acidosis, high altitude. Hydroxyurea = drug of choice (↑HbF). Auto-splenectomy by age 5. Dactylitis = first manifestation in infants. BMT = only cure.

Q15. Which IV fluid is CONTRAINDICATED to run concurrently with blood transfusion?

• A) 0.9% Normal Saline
• B) 5% Dextrose (D5W)
• C) Ringer's Lactate
• D) 0.45% Normal Saline

💡 Tip: Dextrose (D5W) = CONTRAINDICATED with blood — causes RBC clumping and haemolysis (hypo-osmotic + glucose causes swelling of RBCs). Only 0.9% Normal Saline is compatible with blood. Also avoid: Lactated Ringer's (calcium → clot formation), medications in blood bag.

Q16. The smear finding PATHOGNOMONIC of Chronic Lymphocytic Leukemia (CLL) is?

• A) Auer rods
• B) Hypersegmented neutrophils
• C) Smudge cells (Basket cells)
• D) Reed-Sternberg cells

💡 Tip: Smudge/Basket cells = fragile lymphocytes that rupture during smear preparation = HALLMARK of CLL. CLL = elderly, indolent, watch-and-wait, most common leukemia in West. Auer rods = AML. Hypersegmented neutrophils = Megaloblastic anaemia. Reed-Sternberg = Hodgkin's Lymphoma.

Q17. The "hair-on-end" skull X-ray appearance is characteristic of?

• A) Multiple Myeloma
• B) Sickle Cell Anaemia only
• C) Thalassemia Major (Beta Thalassemia)
• D) Aplastic Anaemia

💡 Tip: "Hair-on-end" skull X-ray = Thalassemia major (extramedullary haematopoiesis in diploë expands skull bones). Also known as: Crew-cut appearance. Chipmunk facies + hepatosplenomegaly + growth retardation = other signs. Desferrioxamine = iron chelation for transfusion-related iron overload.

Q18. TRALI (Transfusion-Related Acute Lung Injury) is differentiated from TACO (Transfusion-Associated Circulatory Overload) by?

• A) TRALI occurs later than TACO
• B) TRALI responds better to diuretics
• C) TRALI presents with hypotension; TACO presents with hypertension and raised JVP
• D) TACO occurs only in elderly patients

💡 Tip: TRALI = hypotension + bilateral lung infiltrates (immune-mediated) — NO diuretics, supportive/ventilatory care. TACO = hypertension + raised JVP + bilateral crackles (fluid overload) — GIVE diuretics. TRALI is the leading cause of transfusion-related DEATH. Both occur within 6 hours of transfusion.

⚡ Quick Reference – Blood & Blood Disorders

Most Common Anaemia

IDA (Iron Deficiency)

IDA Earliest Marker

↓ Serum Ferritin

Megaloblastic Hallmark

Hypersegmented neutrophils

Universal Donor

O Negative

Universal Recipient

AB Positive

Most Common Childhood Leukemia

ALL (TdT positive)

AML Hallmark

Auer Rods

CML Chromosome

Philadelphia t(9;22)

CLL Hallmark

Smudge Cells

Hodgkin's Cell

Reed-Sternberg (Owl eye)

Multiple Myeloma

CRAB + Bence-Jones protein

PV Sign

Aquagenic pruritus + JAK2

DIC Sensitive Marker

D-dimer ↑

Hemophilia A

Factor VIII ↑aPTT, nl PT

ITP Finding

↓Platelets nl PT/aPTT

SCA Drug

Hydroxyurea (↑HbF)

Thalassemia X-ray

Hair-on-end skull

PDA Drug (close)

Stop first in any reaction

Transfusion Reaction #1

STOP transfusion first always

WBC Mnemonic

Never Let Monkeys Eat Bananas