Hematopathology Practice Questions

Q: Which of the following is a marker for Langerhans cell histiocytosis?

CD 1a. **Explanation:** **Langerhans Cell Histiocytosis (LCH)** is a clonal proliferation of Langerhans cells, which are specialized dendritic cells normally found in the skin. The diagnosis relies on identifying these cells through specific immunophenotypic markers and ultrastructural findings [1]. **Why CD1a is correct:** Langerhans cells are characterized by the expression of **CD1a** and **Langerin (CD207)**. CD1a is a glycoprotein structurally related to MHC molecules and is highly specific for Langerhans cells when found in the context of histiocytic lesions. Another gold-standard marker is **S100** (though it is less specific). **Analysis of Incorrect Options:** * **CD10:** Also known as CALLA (Common Acute Lymphoblastic Leukemia Antigen), it is a marker for pre-B cells and germinal center B-cells. It is used to diagnose **B-ALL** and Follicular Lymphoma. * **CD30:** A marker for activated T and B cells. It is the hallmark of **Hodgkin Lymphoma** (Reed-Sternberg cells) and **Anaplastic Large Cell Lymphoma (ALCL)**. * **CD56:** An adhesion molecule used as a marker for **Natural Killer (NK) cells** and certain neuroendocrine tumors. **High-Yield Clinical Pearls for NEET-PG:** * **Electron Microscopy:** The pathognomonic finding in LCH is the **Birbeck Granule**, which has a characteristic "tennis-racket" appearance [1]. * **Langerin (CD207):** This is the most specific marker for LCH as it is directly associated with the formation of Birbeck granules [1]. * **Clinical Triad (Hand-Schüller-Christian disease):** Calvarial bone defects, exophthalmos, and diabetes insipidus. * **BRAF V600E Mutation:** Seen in approximately 50% of LCH cases [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, pp. 629-630.

Q: Auto-splenectomy is associated with which of the following conditions?

Systemic lupus erythematosus. **Explanation:** **Auto-splenectomy** refers to the progressive fibrosis and atrophy of the spleen due to repeated vascular insults, eventually leading to a non-functional, shrunken organ. **Why A is correct:** While **Sickle Cell Anemia** is the most common cause of auto-splenectomy (due to repeated micro-infarctions) [1], **Systemic Lupus Erythematosus (SLE)** is a well-recognized cause in adults. In SLE, the mechanism involves vasculitis of the splenic vessels and the deposition of immune complexes, leading to infarction and subsequent atrophy. A characteristic histological finding in SLE is **"onion-skin" thickening** of the splenic penicilliary arteries due to concentric perivascular fibrosis. **Why the other options are incorrect:** * **B. Trauma:** Splenic trauma typically leads to surgical splenectomy (splenectomy due to rupture) or the formation of accessory splenic tissue (splenosis), but not the gradual physiological atrophy known as auto-splenectomy. * **C. Sarcoidosis:** This condition typically causes **splenomegaly** (enlargement) due to the infiltration of non-caseating granulomas. * **D. ITP:** In ITP, the spleen is the site of platelet destruction, but it usually remains **normal in size** or shows only mild enlargement. It does not undergo atrophy. **High-Yield Clinical Pearls for NEET-PG:** * **Most common cause of auto-splenectomy:** Sickle Cell Anemia (usually complete by childhood) [1]. * **Howell-Jolly bodies:** These nuclear remnants in RBCs are a hallmark of a non-functional spleen (seen after auto-splenectomy) [1]. * **Onion-skin lesion:** Pathognomonic splenic finding in SLE. * **Other causes of small spleen:** Celiac disease and Essential Thrombocythemia. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, pp. 631-632.

Q: Which disease is transmitted by all components of blood?

Malaria. **Explanation:** **Malaria (Correct Answer):** Malaria is caused by *Plasmodium* species, which reside within red blood cells (RBCs) during the asexual stage of their life cycle [1]. However, transmission is not limited to packed RBCs. The parasites can be present in any blood component that contains even trace amounts of RBCs or contaminated plasma, including Whole Blood, Packed RBCs, Platelets, and Fresh Frozen Plasma (FFP). Because *Plasmodium* can survive at 4°C for up to 2–3 weeks, it remains viable in standard blood bank storage, making it transmissible via all components. **Incorrect Options:** * **Syphilis (*Treponema pallidum*):** The spirochete is highly sensitive to cold. It cannot survive more than 72 hours (3 days) at 4°C. Since most blood components are refrigerated, the risk of transmission is negligible in stored blood. * **Toxoplasma (*T. gondii*):** While it can be transmitted via organ transplantation or leukocyte-rich products, it is not a standard risk across all components (like FFP) as it primarily resides in nucleated cells. * **H. pylori:** This is a gastrointestinal pathogen transmitted via the fecal-oral route; it is not a blood-borne pathogen. **Clinical Pearls for NEET-PG:** * **Storage Lesion:** Malaria is the classic example of a parasite that survives refrigeration. * **Window Period:** The most common cause of post-transfusion hepatitis is Hepatitis B (due to the window period), but the most common “parasitic” post-transfusion infection is Malaria. * **TTIs (Transfusion-Transmitted Infections):** Mandatory screening in India includes HIV, HBV, HCV, Syphilis, and Malaria. * **Bacterial Contamination:** Platelets are the most likely component to cause bacterial sepsis because they are stored at room temperature (20–24°C).

Q: All of the following investigation findings about Haemophilia A are true, EXCEPT:

PT increased. Hemophilia A is an X-linked recessive bleeding disorder characterized by a deficiency of **Factor VIII** [1]. To answer this question, one must understand the coagulation cascade and which laboratory tests evaluate specific pathways. **Why Option B is the Correct Answer (The Exception):** * **Prothrombin Time (PT)** measures the **Extrinsic** and **Common** pathways (Factors VII, X, V, II, and I) [2]. * Since Hemophilia A involves a deficiency in Factor VIII (a component of the **Intrinsic** pathway), the PT remains **normal** [2]. An increased PT would suggest a deficiency in Factor VII, Vitamin K deficiency, or liver disease. **Analysis of Other Options:** * **A. PTT increased:** Partial Thromboplastin Time (PTT/aPTT) measures the **Intrinsic** and Common pathways (Factors XII, XI, IX, VIII, X, V, II, I) [2]. A deficiency in Factor VIII directly leads to a prolonged aPTT. * **C. Clotting time is increased:** Clotting time is a non-specific measure of the intrinsic pathway. In severe hemophilia, the time taken for whole blood to clot is prolonged. * **D. Serum levels of factor VIII are decreased:** This is the definitive biochemical hallmark of Hemophilia A. **High-Yield Clinical Pearls for NEET-PG:** * **Mixing Study:** If aPTT is prolonged, a mixing study is performed [2]. If the aPTT corrects with normal plasma, it indicates a **factor deficiency** (like Hemophilia); if it doesn't correct, it indicates a **factor inhibitor**. * **Hemophilia B:** Deficiency of Factor IX (Christmas disease); lab findings are identical to Hemophilia A. * **von Willebrand Disease (vWD):** Often confused with Hemophilia A because vWF stabilizes Factor VIII [3]. However, in vWD, the **Bleeding Time is increased** and Ristocetin cofactor assay is abnormal [3]. * **Most common site of bleeding:** Hemarthrosis (bleeding into joints, most commonly the knee).

Q: Bence-Jones proteins are a laboratory finding of:

Multiple myeloma. **Explanation:** **Multiple Myeloma (Option B)** is a plasma cell dyscrasia characterized by the monoclonal proliferation of plasma cells [1]. These cells produce excessive amounts of monoclonal (M) proteins. **Bence-Jones proteins** are specifically the **monoclonal free light chains** (either kappa or lambda) that are small enough to be filtered by the glomerulus and excreted in the urine [2]. A key biochemical property is their unique solubility: they precipitate when heated to 40–60°C and redissolve upon boiling (100°C). **Why other options are incorrect:** * **Paget’s Disease (Option A):** A disorder of bone remodeling characterized by increased osteoclastic and osteoblastic activity. Key markers include elevated Serum Alkaline Phosphatase (ALP) and urinary hydroxyproline, not light chains. * **Malignant Melanoma (Option C):** A melanocytic skin cancer. While it may have various tumor markers (like S100 or LDH for prognosis), it does not involve immunoglobulin production. * **Fibrous Dysplasia (Option D):** A benign bone condition where normal bone is replaced by fibrous tissue (showing a "ground-glass" appearance on X-ray). It does not involve plasma cell pathology. **High-Yield Clinical Pearls for NEET-PG:** * **Diagnosis:** Bence-Jones proteins are detected by **Urine Protein Electrophoresis (UPEP)** or Immunofixation [2]. They are *not* detected by standard urine dipsticks (which primarily sense albumin). * **Renal Impact:** These light chains are nephrotoxic and lead to **"Myeloma Kidney"** (Cast Nephropathy). * **CRAB Criteria for Myeloma:** **C**alcium elevation, **R**enal insufficiency, **A**nemia, and **B**one lesions (lytic "punched-out" lesions) [2]. * **Diagnostic Hallmark:** Bone marrow biopsy showing >10% clonal plasma cells [2].

Question 1

Which of the following is a marker for Langerhans cell histiocytosis?

Accepted Answer

CD 1a

Answer

CD 10

Answer

CD 30

Answer

CD 56

Question 2

Which of the following stains is used?

Accepted Answer

Brilliant cresyl blue

Answer

Methyl violet

Answer

Sudan black

Answer

Indigo carmine

Question 3

Auto-splenectomy is associated with which of the following conditions?

Accepted Answer

Systemic lupus erythematosus

Answer

Trauma

Answer

Sarcoidosis

Answer

Idiopathic thrombocytopenic purpura (ITP)

Question 4

As compared to iron deficiency anemia, which of the following is decreased in anemia of chronic disease?

Accepted Answer

Total iron-binding capacity (TIBC)

Answer

Endogenous bone marrow iron stores

Answer

Serum ferritin

Answer

Transferrin saturation

Question 5

Which disease is transmitted by all components of blood?

Accepted Answer

Malaria

Answer

Syphilis

Answer

Toxoplasma

Answer

H. pylori

Question 6

Which of the following is not a feature of multiple myeloma?

Accepted Answer

t(8;14) translocation

Answer

Recurrent infection

Answer

Neurological manifestation

Answer

Hypercalcemia

Question 7

Deficiency of GpIIb-IIIa results in which of the following conditions?

Accepted Answer

Glanzmann thrombasthenia

Answer

Wiskott-Aldrich syndrome

Answer

Bernard-Soulier syndrome

Answer

None of the above

Question 8

Which of the following cell types is NOT typically found in myelodysplastic syndrome?

Accepted Answer

Megakaryocytes with normal single multilobulated nuclei

Answer

Ringed sideroblasts

Answer

Pseudo-Pelger-Huet cells

Answer

Pawn ball megakaryocytes

Question 9

All of the following investigation findings about Haemophilia A are true, EXCEPT:

Accepted Answer

PT increased

Answer

PTT increased

Answer

Clotting time is increased

Answer

Serum levels of factor VIII are decreased

Question 10

Bence-Jones proteins are a laboratory finding of:

Accepted Answer

Multiple myeloma

Answer

Paget's disease

Answer

Malignant melanoma

Answer

Fibrous dysplasia

Hematopathology — MCQs

Hematopathology — MCQs

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