Q5
LONG ANSWER
10M
★ High-yield
Describe the approach to a patient with a bleeding disorder. Interpret the coagulation screening tests and classify the causes of thrombocytopenia.
The Three Components of Haemostasis
| Component | Role | Disorders |
|---|---|---|
| Vessel wall | Vasoconstriction; provides collagen and von Willebrand factor | Scurvy, senile purpura, Henoch–Schonlein purpura, hereditary haemorrhagic telangiectasia, steroid purpura |
| Platelets | Primary haemostatic plug | Thrombocytopenia; defects of function |
| Coagulation factors | Fibrin stabilisation of the plug | Haemophilia, von Willebrand disease, liver disease, vitamin K deficiency, DIC |
Platelet Compared with Coagulation Bleeding
| Feature | Platelet / vessel disorder | Coagulation disorder |
|---|---|---|
| Onset after injury | Immediate | Delayed, hours later |
| Site | Superficial — skin and mucous membranes | Deep — joints, muscles, retroperitoneum |
| Characteristic lesion | Petechiae and purpura; epistaxis, gum bleeding, menorrhagia | Haemarthrosis and muscle haematoma |
| Petechiae | Typical | Rare |
| Bleeding after minor cuts | Persistent oozing | Minimal |
| Family history | Usually absent | Often present (X-linked in haemophilia) |
| Sex | Both | Males in haemophilia |
| Example | ITP | Haemophilia |
CLINICAL PEARL
- The history usually tells you which system has failed before any test is done.
- Immediate, superficial, mucocutaneous bleeding is a platelet or vessel problem
- delayed, deep bleeding into joints and muscles is a coagulation problem. Taking that history properly directs the whole investigation and avoids ordering everything at once.
Screening Tests and Their Interpretation
| Test | Measures | Normal |
|---|---|---|
| Platelet count | Number of platelets | 1.5–4 lakh/mm3 |
| Bleeding time | Platelet number and function, and vessel wall | 2–7 minutes |
| Prothrombin time (PT/INR) | Extrinsic and common pathway — factors VII, X, V, II, I | 11–16 seconds |
| Activated partial thromboplastin time (aPTT) | Intrinsic and common pathway — XII, XI, IX, VIII, X, V, II, I | 25–40 seconds |
| Thrombin time | Fibrinogen to fibrin conversion | 10–15 seconds |
| Fibrinogen and D-dimer | Consumption and fibrinolysis | — |
| PT | APTT | Platelets | Diagnosis |
|---|---|---|---|
| Prolonged | Normal | Normal | Factor VII deficiency; early warfarin or vitamin K deficiency; early liver disease |
| Normal | Prolonged | Normal | Haemophilia A (VIII) or B (IX); von willebrand disease; heparin; lupus anticoagulant |
| Prolonged | Prolonged | Normal | Vitamin K deficiency; liver disease; common pathway defect |
| Prolonged | Prolonged | Low | DIC; massive transfusion; severe liver disease |
| Normal | Normal | Low | ITP; hypersplenism; marrow failure |
| Normal | Normal | Normal | Vessel wall disorder, or a platelet function defect (check bleeding time) |
Causes of Thrombocytopenia
| Mechanism | Causes |
|---|---|
| Decreased production | Aplastic anaemia, leukaemia, marrow infiltration, megaloblastic anaemia, alcohol, cytotoxic drugs, radiation |
| Increased destruction — immune | ITP; SLE; CLL and lymphoma; drugs (heparin, quinine, sulphonamides); post-transfusion purpura; HIV |
| Increased destruction — non-immune | DIC, thrombotic thrombocytopenic purpura, haemolytic uraemic syndrome, dengue and other viral infections, malaria, sepsis |
| Sequestration | Hypersplenism from any cause of splenomegaly |
| Dilutional | Massive transfusion |
| Spurious | PSEUDOthrombocytopenia from EDTA-induced clumping — always check the film before acting |
- In India, dengue is a very common cause of acute thrombocytopenia, and the count often falls dramatically before recovering spontaneously
- Spontaneous bleeding is unusual above 20,000/mm3, and serious bleeding above 10,000, unless platelet function is also impaired
Vitamin K Deficiency and Liver Disease
| Feature | Vitamin K deficiency | Liver disease |
|---|---|---|
| Factors affected | II, VII, IX, X and proteins C and S only | All factors except VIII and von Willebrand factor |
| Factor VIII | Normal | Normal or raised — it is made by endothelium, not hepatocytes |
| Factor V | Normal | Low |
| Response to vitamin K | Corrects | Little or no correction |
| Causes | Newborn (haemorrhagic disease), obstructive jaundice, malabsorption, prolonged antibiotics, warfarin | Cirrhosis, acute hepatic failure |
- Factor V distinguishes them — it is made in the liver but is not vitamin K dependent, so it is normal in vitamin K deficiency and low in liver disease
Applied Aspects
- A careful history is the most valuable investigation — family history, drugs, response to previous surgery and dental extraction, and the pattern of bleeding
- Vitamin K at birth prevents haemorrhagic disease of the newborn, and is among the most cost-effective interventions in medicine
- Platelet transfusion is given for bleeding or before a procedure, not for a number alone; it is contraindicated in thrombotic thrombocytopenic purpura, where it fuels the thrombosis
- Always examine the film in unexplained thrombocytopenia — it excludes clumping, and may show schistocytes, blasts or malarial parasites
- A prolonged aPTT that corrects on mixing with normal plasma indicates a factor deficiency; one that does not correct indicates an inhibitor, such as a lupus anticoagulant or an acquired factor VIII antibody
- Aspirin and NSAIDs must be stopped before surgery in any patient with a bleeding tendency, and should never be used for fever in suspected dengue
All questions in Haematology II: Leukaemias, Lymphomas & Haemostasis
- Q1Classify leukaemias. Describe the clinical features and laboratory diagnosis of acute leukaemia and differentiate AML from ALL. ★
- Q2Describe chronic myeloid leukaemia and chronic lymphocytic leukaemia with their clinical features and laboratory findings. ★
- Q3Classify lymphomas. Describe Hodgkin lymphoma and differentiate it from non-Hodgkin lymphoma. ★
- Q4Describe the clinical features, laboratory diagnosis and complications of multiple myeloma. ★
- Q5Describe the approach to a patient with a bleeding disorder. Interpret the coagulation screening tests and classify the causes of thrombocytopenia. ★
- SN1Describe immune thrombocytopenic purpura with its types, features and management. ★
- SN2Describe haemophilia with its genetics, clinical features and laboratory diagnosis. ★
- SN3Describe von Willebrand disease and differentiate it from haemophilia A. ★
- SN4Describe the Reed-Sternberg cell with its variants and immunophenotype. ★
- SN5Define leukaemoid reaction. Differentiate it from chronic myeloid leukaemia and add a note on the leucoerythroblastic reaction. ★
- SN6Describe the indications, sites and interpretation of bone marrow examination. ★
- SN7Describe the hazards of blood transfusion.