Hematopathology Practice Questions

Q: What is the shelf life of blood stored in CPDA buffer in a blood bank?

35 days. **Explanation:** The shelf life of stored blood is determined by the anticoagulant-preservative solution used, which maintains red cell viability and function by providing glucose for metabolism and preventing coagulation. **1. Why C is correct:** **CPDA-1 (Citrate Phosphate Dextrose Adenine)** is the most commonly used preservative in blood banks. The addition of **Adenine** is the key factor here; it acts as a substrate for ATP synthesis, which maintains the red cell membrane integrity and extends the shelf life to **35 days**. Without adenine (as in CPD), the shelf life is significantly shorter. **2. Why the other options are incorrect:** * **A (21 days):** This is the shelf life for older preservatives like **ACD (Acid Citrate Dextrose)** and **CPD (Citrate Phosphate Dextrose)**. These lack adenine, leading to faster ATP depletion. * **B (30 days):** This is not a standard shelf life for any conventional blood bag preservative. * **D (42 days):** This is the shelf life for blood stored with **Additive Solutions (SAGM - Saline, Adenine, Glucose, and Mannitol)**. In this system, the plasma is removed, and the additive solution is added to the packed red cells to extend viability. **3. High-Yield Clinical Pearls for NEET-PG:** * **Storage Temperature:** Whole blood and PRBCs must be stored at **2°C to 6°C**. * **The "Storage Lesion":** During storage, there is a decrease in 2,3-DPG (shifting the oxygen dissociation curve to the left), a decrease in pH, and an **increase in extracellular Potassium** (important in neonatal transfusions). * **Platelet Storage:** Unlike RBCs, platelets are stored at **20°C to 24°C** with constant agitation and have a shelf life of only **5 days**. * **FFP Storage:** Frozen at **-30°C or colder**, FFP has a shelf life of **1 year**.

Q: What is the most common mutation found in hereditary elliptocytosis?

Spectrin. **Explanation:** Hereditary Elliptocytosis (HE) is an autosomal dominant red blood cell membrane disorder characterized by the presence of elliptical or cigar-shaped erythrocytes. The primary defect lies in the **horizontal interactions** of the cytoskeleton, which leads to mechanical instability of the RBC membrane. **Why Spectrin is correct:** The most common molecular defect in HE involves mutations in **α-spectrin** (specifically the SPTA1 gene), followed by β-spectrin. These mutations typically disrupt the formation of **spectrin dimers into tetramers**. When the spectrin scaffold cannot properly assemble or cross-link, the cell fails to recoil to its biconcave shape after passing through microcapillaries, resulting in permanent elliptical deformation. **Why other options are incorrect:** * **Ankyrin:** Mutations in Ankyrin are the most common cause of **Hereditary Spherocytosis (HS)**, affecting the *vertical* stability between the skeleton and the lipid bilayer [1]. * **Glycophorin A:** While Glycophorin C mutations can cause a rare subtype of HE (Leach phenotype), Glycophorin A is primarily a blood group antigen carrier and not the most common site for HE mutations. * **Band 4.2:** Deficiency of Protein 4.2 is a known but less common cause of Hereditary Spherocytosis [1], particularly prevalent in the Japanese population. **High-Yield Clinical Pearls for NEET-PG:** * **HE Hallmark:** Defect in **Horizontal interactions** (Spectrin tetramer assembly). * **HS Hallmark:** Defect in **Vertical interactions** (Ankyrin/Band 3/Spectrin binding to membrane) [1]. * **Blood Smear:** >25% elliptocytes is diagnostic for HE. * **Southeast Asian Ovalocytosis (SAO):** A specific subtype of HE caused by a mutation in **Band 3**, providing protection against malaria. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 640-641.

Q: Dohle-like inclusions in neutrophils along with giant platelets is characteristic of which of the following conditions?

May-Hegglin anomaly. **Explanation:** **May-Hegglin Anomaly (MHA)** is an autosomal dominant disorder characterized by a triad of **thrombocytopenia, giant platelets, and large, basophilic leukocyte inclusions** (Döhle-like bodies). 1. **Why it is correct:** The condition is caused by a mutation in the **MYH9 gene**, which encodes the non-muscle myosin heavy chain IIA. This defect leads to disordered megakaryocyte maturation (resulting in giant platelets) and the precipitation of myosin heavy chains in the cytoplasm of neutrophils, forming **Döhle-like bodies**. Unlike true Döhle bodies (seen in infections/burns), these are larger, more prominent, and persist even in the absence of inflammation. 2. **Why other options are incorrect:** * **Bernard-Soulier Syndrome:** Features giant platelets and thrombocytopenia (due to GpIb-IX-V deficiency) [1], but **lacks** leukocyte inclusions. * **Chédiak-Higashi Syndrome:** Characterized by **massive lysosomal granules** in leukocytes and partial albinism, not Döhle-like bodies or giant platelets. * **Pelger-Huët Anomaly:** Involves a failure of nuclear segmentation (hyposegmented, "pince-nez" neutrophils) but does not affect platelets. **High-Yield Clinical Pearls for NEET-PG:** * **MYH9-Related Disorders:** MHA is part of a spectrum including Epstein and Fechtner syndromes (which add nephritis and sensorineural hearing loss to the hematologic findings). * **Döhle-like vs. Döhle bodies:** True Döhle bodies are composed of **rough endoplasmic reticulum**; Döhle-like bodies in MHA are composed of **myosin heavy chains**. * **Peripheral Smear:** Always look for the combination of "Big Platelets + Blue Inclusions" to diagnose May-Hegglin. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 668-669.

Q: Which of the following is a common bleeding manifestation of hemophilia?

Hemarthrosis. **Explanation:** Hemophilia (A and B) is a disorder of **secondary hemostasis** caused by deficiencies in clotting factors VIII and IX, respectively [1]. Unlike primary hemostatic defects (platelet disorders), which present with superficial mucosal bleeding, secondary hemostatic defects manifest as deep-seated bleeding. **Why Hemarthrosis is Correct:** **Hemarthrosis** (bleeding into joint spaces) is the hallmark clinical manifestation of hemophilia [1]. It occurs most commonly in the weight-bearing joints (knees, elbows, and ankles). Repeated episodes lead to synovial hypertrophy and chronic joint destruction, known as hemophilic arthropathy [1]. **Analysis of Incorrect Options:** * **Hemoptysis (A):** Bleeding from the respiratory tract is rare in hemophilia and usually suggests underlying pulmonary pathology (e.g., TB or malignancy) rather than a primary coagulation defect. * **Hematomas (C):** While large, deep muscle hematomas are common in hemophilia, they are generally considered less specific or "classic" than hemarthrosis [1]. In the context of a "most common/hallmark" question, hemarthrosis is the preferred answer. * **Hematuria (D):** Though it can occur in hemophiliacs, it is not the primary or most characteristic presenting feature [1]. **NEET-PG High-Yield Pearls:** * **Inheritance:** Both Hemophilia A and B are **X-linked recessive**. * **Lab Findings:** Characterized by a **prolonged aPTT** with a **normal PT and Bleeding Time (BT)**. * **Mixing Study:** The prolonged aPTT will **correct** upon mixing with normal plasma (differentiating it from factor inhibitors). * **Treatment:** Factor replacement is the mainstay. For mild Hemophilia A, **Desmopressin (DDAVP)** can be used as it releases stored Factor VIII and vWF from Weibel-Palade bodies. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 623-624.

Question 1

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

Accepted Answer

Elevated alkaline phosphatase

Answer

Hypercalcemia

Answer

Anemia

Answer

Hyperviscosity

Question 2

What is the shelf life of blood stored in CPDA buffer in a blood bank?

Accepted Answer

35 days

Answer

21 days

Answer

30 days

Answer

42 days

Question 3

Cytogenetic studies in a 70-year-old woman with chronic myelogenous leukemia (CML) demonstrate a t(9;22) chromosomal translocation. Which of the following best explains the role of this translocation in the pathogenesis of leukemia in this patient?

Accepted Answer

Protooncogene activation

Answer

Altered DNA methylation status

Answer

Enhanced expression of telomerase gene

Answer

Expansion of a trinucleotide repeat

Question 4

What is the most common mutation found in hereditary elliptocytosis?

Accepted Answer

Spectrin

Answer

Ankyrin

Answer

Glycophorin A

Answer

Band 4.2

Question 5

Which cells are characteristic of Hodgkin's disease?

Accepted Answer

Reed-Sternberg cells

Answer

Lacunar cells

Answer

Giant cells

Answer

Eosinophils

Question 6

A 23-year-old female presented with palpable lumps in the axilla and supra-clavicular region. Following investigations including X-ray, USG-guided biopsy, and HPE, a diagnosis was made. Flow cytometry revealed CD15 and CD30 positivity. She was started on chemotherapy. Later, the patient developed fatigue, dyspnea, and orthopnea, with ECG showing left ventricular hypertrophy. Which of the following chemotherapeutic agents is most likely responsible for these manifestations?

Accepted Answer

Bleomycin

Answer

Daunorubicin

Answer

Capecitabine

Answer

Cyclophosphamide

Question 7

Dohle-like inclusions in neutrophils along with giant platelets is characteristic of which of the following conditions?

Accepted Answer

May-Hegglin anomaly

Answer

Bernard-Soulier syndrome

Answer

Chédiak-Higashi syndrome

Answer

Pelger-Huët anomaly

Question 8

Which of the following is a common bleeding manifestation of hemophilia?

Accepted Answer

Hemarthrosis

Answer

Hemoptysis

Answer

Hematomas

Answer

Hematuria

Question 9

Multiple myeloma is characterized by all of the following except?

Accepted Answer

Polyclonal gammopathy

Answer

Presence of light chains

Answer

Monoclonal gammopathy

Answer

Hypergammaglobulinemia

Question 10

Ringed sideroblasts are seen in which of the following conditions?

Accepted Answer

Myelodysplastic syndrome

Answer

Iron deficiency anemia

Answer

Thalassemia

Answer

Anemia of chronic disease

Hematopathology — MCQs

Hematopathology — MCQs

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