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 current permissible maximum storage time and temperature for platelets?

5 days at 20-24°C. **Explanation** The correct answer is **B. 5 days at 20-24°C**. **1. Understanding the Concept** Platelets are unique compared to other blood components because they must be stored at **room temperature (20-24°C)** with **continuous agitation**. Agitation prevents platelet aggregation and maintains oxygen exchange, which is vital for preserving the "platelet storage lesion" (metabolic viability). If platelets are refrigerated (1-6°C), they undergo irreversible structural changes and are rapidly cleared by the liver upon transfusion, rendering them ineffective. The storage limit is capped at **5 days** primarily due to the high risk of bacterial proliferation at room temperature. **2. Analysis of Incorrect Options** * **Option A (1-6°C):** This is the storage temperature for Whole Blood and Packed Red Blood Cells (PRBCs). Cold temperatures cause platelet activation and dysfunction. * **Option C (7 days):** While some specialized bags or pathogen-reduction technologies (PRT) allow for 7-day storage in certain countries, the standard global and Indian (DGHS/NBTC) guideline remains 5 days to minimize sepsis risk. * **Option D (25-27°C):** This temperature is too high and exceeds the physiological range, accelerating bacterial growth and platelet degradation. **3. High-Yield Clinical Pearls for NEET-PG** * **Agitation:** Must be continuous (using a platelet agitator). * **Dose:** 1 unit of Random Donor Platelets (RDP) increases the count by **5,000–10,000/µL**; 1 unit of Single Donor Platelets (SDP) increases it by **30,000–60,000/µL**. * **Transfusion Trigger:** Usually <10,000/µL in stable patients or <50,000/µL if undergoing surgery. * **Complication:** Platelets have the **highest risk of bacterial contamination** among all blood products.

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: A 15-year-old boy presented with a 1-day history of bleeding gums, subconjunctival bleed, and purpuric rash. Investigations revealed: Hb 6.4 gm/dL; TLC-26,500/mm3; prothrombin time-20 sec with a control of 13 sec; partial thromboplastin time-50 sec; and fibrinogen 10 mg/dL. Peripheral smear was suggestive of acute myeloblastic leukemia. Which of the following is the most likely diagnosis?

Promyelocytic leukemia. ### Explanation The clinical presentation of a young patient with sudden onset bleeding (gums, subconjunctival, purpura) combined with a high white cell count and profound coagulopathy is classic for **Acute Promyelocytic Leukemia (APL)**, formerly classified as FAB M3 [1], [3]. **1. Why Promyelocytic Leukemia is Correct:** The hallmark of APL is the association with **Disseminated Intravascular Coagulation (DIC)** [2]. This is evidenced in the question by the prolonged PT (20s), prolonged PTT (50s), and critically low fibrinogen (10 mg/dL; normal: 200–400 mg/dL). In APL, the primary granules of the malignant promyelocytes contain procoagulants and fibrinolytic enzymes (like annexin II) that trigger a massive consumptive coagulopathy upon release. **2. Why Other Options are Incorrect:** * **A & B (M1 & M2):** While these are common subtypes of AML, they typically present with signs of bone marrow failure (anemia, infections) [1]. While they can cause thrombocytopenia, they rarely present with such severe, primary DIC at the time of diagnosis. * **D (M4):** Myelomonocytic leukemia is often associated with extramedullary involvement (e.g., gingival hyperplasia or CNS involvement) rather than the severe consumptive coagulopathy seen here [3]. **3. High-Yield Pearls for NEET-PG:** * **Cytogenetics:** Associated with **t(15;17)**, involving the **PML-RARA** fusion gene. * **Morphology:** Characterized by **Auer rods** (often in bundles called **Faggot cells**). * **Treatment:** Medical emergency! Start **ATRA (All-trans retinoic acid)** or Arsenic Trioxide immediately to differentiate the blasts and resolve DIC. * **Complication:** Watch for **Differentiation Syndrome** (fever, dyspnea, pulmonary infiltrates) during treatment.

Q: Small platelets on peripheral smear examination are seen in which of the following conditions?

Wiskott-Aldrich syndrome. **Explanation:** The size of platelets on a peripheral smear is a critical diagnostic clue in pediatric hematology. **1. Why Wiskott-Aldrich Syndrome (WAS) is correct:** WAS is an X-linked recessive disorder caused by mutations in the *WAS* gene, which encodes the Wiskott-Aldrich Syndrome Protein (WASP). This protein is essential for actin cytoskeleton remodeling. Defects lead to the formation of **microthrombocytes** (small platelets) due to abnormal megakaryocyte proplatelet formation and increased splenic clearance. It is classically characterized by the triad of **Thrombocytopenia (with small platelets), Eczema, and Recurrent infections** (due to combined B and T cell deficiency). **2. Why the other options are incorrect:** * **Bernard-Soulier Syndrome (BSS):** Characterized by a deficiency of GP Ib-IX-V complex. It is the classic cause of **Giant Platelets** (often as large as red blood cells) and failure of platelet aggregation with Ristocetin. * **Gray Platelet Syndrome:** A rare alpha-granule deficiency. Platelets appear large, pale, and "ghost-like" (gray) on Wright-Giemsa stain due to the lack of granules. * **Von Willebrand Disease (vWD) Type 2B:** This "gain-of-function" mutation leads to increased binding of vWF to GP Ib, causing spontaneous platelet aggregation and clearance. This typically results in mild **thrombocytopenia with large/giant platelets** (due to increased turnover). **High-Yield Clinical Pearls for NEET-PG:** * **Small Platelets:** Wiskott-Aldrich Syndrome, X-linked Thrombocytopenia (XLT). * **Giant Platelets:** Bernard-Soulier Syndrome, May-Hegglin Anomaly (look for Dohle-like bodies in neutrophils), and ITP (due to young, "stress" platelets). * **WAS Mnemonic:** **W**-**A**-**S** (Small platelets, Aldrich, Sex-linked/X-linked). * **Treatment of choice for WAS:** Hematopoietic stem cell transplant (HSCT).

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

What is the current permissible maximum storage time and temperature for platelets?

Accepted Answer

5 days at 20-24°C

Answer

5 days at 1-6°C

Answer

7 days at 22-24°C

Answer

5 days at 25-27°C

Question 4

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 5

What is the most common mutation found in hereditary elliptocytosis?

Accepted Answer

Spectrin

Answer

Ankyrin

Answer

Glycophorin A

Answer

Band 4.2

Question 6

A 15-year-old boy presented with a 1-day history of bleeding gums, subconjunctival bleed, and purpuric rash. Investigations revealed: Hb 6.4 gm/dL; TLC-26,500/mm3; prothrombin time-20 sec with a control of 13 sec; partial thromboplastin time-50 sec; and fibrinogen 10 mg/dL. Peripheral smear was suggestive of acute myeloblastic leukemia. Which of the following is the most likely diagnosis?

Accepted Answer

Promyelocytic leukemia

Answer

Myeloblastic leukemia without maturation

Answer

Myeloblastic leukemia with maturation

Answer

Myelomonocytic leukemia

Question 7

A 60-year-old man presented with fatigue, weight loss, and heaviness in the left hypochondrium for 6 months. The hemogram showed Hb 10 gm/dL, TLC 5 lakhs/mm3, platelet count 4 lakhs/mm3, and DLC showed neutrophils 55%, lymphocytes 4%, monocytes 2%, basophils 6%, metamyelocytes 10%, myelocytes 18%, promyelocytes 2%, and blasts 3%. What is the most likely cytogenetic abnormality in this case?

Accepted Answer

t(9;22)

Answer

t(1;21)

Answer

t(15;17)

Answer

Trisomy 21

Question 8

Small platelets on peripheral smear examination are seen in which of the following conditions?

Accepted Answer

Wiskott-Aldrich syndrome

Answer

Von Willebrand disease 2B

Answer

Gray platelet syndrome

Answer

Bernard-Soulier syndrome

Question 9

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 10

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

Accepted Answer

Hemarthrosis

Answer

Hemoptysis

Answer

Hematomas

Answer

Hematuria

Hematopathology — MCQs

Hematopathology — MCQs

On this page

Practice by Chapter

Want unlimited practice?