Hematology Practice Questions

Q: A 16-year-old boy presents with jaundice and splenomegaly. His father had a similar illness during adolescence. MCHC : high? What is the most likely diagnosis?

B. Hereditary spherocytosis. ***Hereditary spherocytosis*** - The presentation of **jaundice**, **splenomegaly**, strong **family history** (autosomal dominant pattern), and an elevated **MCHC** is classic for Hereditary Spherocytosis. [1] - Elevated MCHC is a key diagnostic feature due to mild cellular dehydration in the characteristic **spherocytes**. [1] *IDA* - **Iron Deficiency Anemia (IDA)** is typically microcytic and hypochromic, leading to a **low MCHC**, not high. - Symptoms of IDA include fatigue and pallor, generally without marked jaundice or splenomegaly unless severe. *Thalassemia major* - **Thalassemia major** presents with severe microcytic, hypochromic anemia (low MCHC) usually evident in early infancy, requiring regular transfusions. - While it causes jaundice and hepatosplenomegaly, it is characterized by severe bone changes and a very low MCHC. *AIHA* - **Autoimmune Hemolytic Anemia (AIHA)** typically presents acutely or subacutely and often responds to immunosuppression (e.g., steroids). - Unlike Hereditary Spherocytosis, AIHA generally does not present with a positive **family history** and the primary lab finding is a positive **Direct Antiglobulin Test (DAT)**, not specifically high MCHC.

Q: Which of the following is the most common inherited bleeding disorder?

Von Willebrand Disease. ***Von Willebrand Disease*** - VWD is the most common inherited bleeding disorder, with a prevalence estimated to be as high as 1% of the population, often showing an **autosomal dominant** inheritance pattern. - It results from a quantitative or qualitative defect in **von Willebrand factor (vWF)**, which impairs platelet adhesion and stabilizes **Factor VIII** [1][2]. *Disseminated Intravascular Coagulation* - DIC is an **acquired** life-threatening condition caused by systemic activation of coagulation, usually triggered by underlying severe diseases like **sepsis** or trauma, not inheritance. - It is characterized by simultaneous widespread thrombosis and severe bleeding due to the consumption of clotting factors and platelets. *Factor V Deficiency* - Factor V deficiency (Parahemophilia) is an extremely rare inherited bleeding disorder, transmitted in an **autosomal recessive** manner. - While inherited, its overall prevalence is substantially lower compared to VWD, making it not the most common. *Hemophilia* - Hemophilia (A and B) is the most common severe inherited bleeding disorder, known for causing deep tissue bleeding (e.g., **hemarthroses**). - It is an **X-linked recessive** condition, meaning it is less prevalent overall in the population compared to the high frequency of mild, often undiagnosed, VWD cases.

Q: A 40-year-old patient presents with complains of low back pain and development of pallor. During workup very high levels of urinary beta2 microglobulin was noted. X-ray skull was performed. Bone marrow aspiration shows >10% plasma cells and serum electrophoresis shows M protein >30 g/L. All are useful for management of this patient except?

Melphalan. ***Melphalan*** - While melphalan is used in **multiple myeloma**, its high-dose application is primarily a conditioning regimen for **autologous stem cell transplantation**, rather than a standalone maintenance or initial therapy, especially in younger patients who are transplant-eligible. - Furthermore, alkylating agents like melphalan are associated with an increased risk of **secondary malignancies**, making their long-term use less favorable in transplant-eligible patients when newer, less toxic agents are available. *Lenalidomide* - **Lenalidomide** is an immunomodulatory drug (IMiD) that is highly effective in the treatment of multiple myeloma, often used in maintenance therapy post-transplant or as part of initial induction regimens. - It works by modulating the immune system and has direct anti-myeloma activity, making it a valuable tool in the management of this patient [1]. *Bortezomib* - **Bortezomib** is a proteasome inhibitor that is a cornerstone of initial induction therapy for transplant-eligible and transplant-ineligible multiple myeloma patients [1]. - It effectively inhibits the break down of cellular proteins, leading to apoptosis of myeloma cells, and is crucial for achieving deep responses. *Autologous transplantation* - Given the patient's age (40 years old) and likely fitness, **autologous stem cell transplantation** is a standard and highly effective treatment option for multiple myeloma, aimed at achieving deep and durable remissions [1]. - It involves harvesting the patient's own stem cells, then administering high-dose chemotherapy (often melphalan) to eradicate myeloma cells, followed by reinfusion of the stored stem cells to rescue hematopoiesis [1].

Q: Comment on the diagnosis in the patient shown below?

Hand and foot syndrome. ***Hand and foot syndrome*** - The image shows **swelling and dactylitis** of the fingers, along with a peripheral blood smear demonstrating **sickle cells** (indicated by arrows). This combination is characteristic of hand-foot syndrome, also known as **dactylitis**, which is often the first manifestation of **sickle cell disease** in infants and young children. - **Dactylitis** results from **vaso-occlusion** in the small bones of the hands and feet, leading to painful swelling. *Squaring of metacarpals* - **Squaring of metacarpals** is not a typical finding in sickle cell disease and does not correlate with the observed hand swelling and erythrocyte morphology. - This term is sometimes used to describe changes seen in conditions like **rheumatoid arthritis**, but not in the context of dactylitis associated with sickle cells. *Sub-periosteal resorption* - **Sub-periosteal resorption** is a hallmark radiological finding in **hyperparathyroidism**, affecting bones, typically in the phalanges. - It is not directly suggested by the hand swelling (which is soft tissue/marrow oedema) or the presence of sickle cells in the blood smear. *Peripheral oedema with iron deficiency anemia* - While the picture shows **peripheral oedema** (swelling), the concomitant finding of **sickle cells** in the blood smear strongly indicates **sickle cell disease**, not iron deficiency anemia. - **Iron deficiency anemia** typically presents with microcytic hypochromic red cells, not sickle-shaped cells, and while it can cause fatigue and pallor, it does not directly lead to the dactylitis seen here.

Q: An 8-year-old child of migrant construction worker presents with fever for last 2 weeks with progressive pallor. Complete blood counts reveal pancytopenia, malaria work up is negative, Total serum protein is 7 gm% with Serum Globulin of 4.5 g/dL, serum albumin 2.5 g/dL. Bone marrow study shows?

L.D bodies. ***L.D bodies*** - The image provided shows **macrophages** laden with numerous **Leishman-Donovan bodies**, which are intracellular amastigotes of *Leishmania* parasites. This bone marrow finding, in conjunction with **pancytopenia** and **fever with progressive pallor** in a child from a region where migrant workers live (suggesting possible exposure to endemic diseases), points strongly to **visceral leishmaniasis (kala-azar)**. - The elevated serum globulin and hyperglobulinemia supports visceral leishmaniasis but is not diagnostic, and helps differentiate from conditions like aplastic anemia. *Aplastic crisis* - An aplastic crisis results in **acute red-cell aplasia** with **reticulocytopenia**, characterized by a selective reduction in erythroid precursors rather than generalized pancytopenia. - It would not show the presence of **L.D. bodies** or hypergammaglobulinemia. *Myelofibrosis* - Myelofibrosis is characterized by **fibrotic bone marrow** and usually presents with cytopenias, but also often with **splenomegaly** and a leukoerythroblastic peripheral smear. - The bone marrow would show *fibrosis* and not parasitic inclusions. *Myeloid metaplasia* - Myeloid metaplasia (extramedullary hematopoiesis) is the presence of hematopoietic tissue outside the bone marrow, typically in the spleen or liver, and occurs in response to severe bone marrow dysfunction (e.g., myelofibrosis). - While it can be associated with pancytopenia, the primary bone marrow pathology here is parasitic infection, not a compensatory shift in hematopoiesis.

Q: In a stable, nonbleeding patient with hypoproliferative thrombocytopenia and no additional bleeding risk factors, what is the threshold for prophylactic administration of the following blood product?

$<10,000$. ***<10,000*** - The image clearly shows a bag labeled "PLATELETS." Prophylactic platelet transfusions are generally recommended when the **platelet count drops below 10,000/µL** in hemodynamically stable patients without additional risk factors for bleeding. - This threshold helps prevent **spontaneous hemorrhage**, especially in critically ill patients, as severe thrombocytopenia significantly increases the risk of serious bleeding events like intracranial hemorrhage. *<5,00* - A platelet count of **less than 5,000/µL** is an extremely low threshold, indicating a very high risk of spontaneous bleeding. - While transfusion would certainly be indicated at this level, the typical prophylactic threshold to prevent bleeding in stable patients is higher, at 10,000/µL. *<20,000* - A platelet count of **less than 20,000/µL** is a common threshold for prophylactic platelet transfusion when there are additional risk factors for bleeding, such as fever, sepsis, or certain medications. - However, for general prophylactic transfusion in *stable* patients without these additional risk factors, the lower threshold of <10,000/µL is usually applied. *<50,000* - A platelet count of **less than 50,000/µL** is typically the threshold for prophylactic platelet transfusion in patients who are undergoing (or about to undergo) an **invasive procedure** or surgery where hemostasis is critical. - It is not the general prophylactic threshold for preventing spontaneous bleeding in stable, non-surgical patients.

Q: A patient in ICU was given blood transfusion. 3 hours later SpO2 is reduced to 75% with respiratory difficulty. CVP is 15 cm water and PCWP is 25 mm Hg . CXR is shown below. What is the diagnosis?

Transfusion associated circulatory overload. ***Transfusion associated circulatory overload*** - The combination of **respiratory difficulty**, **reduced SpO2**, **elevated CVP (15 cm water)**, and **markedly elevated PCWP (25 mm Hg)** after a blood transfusion strongly indicates hydrostatic pulmonary edema due to fluid overload. - The CXR image showing **diffuse bilateral infiltrates** (pulmonary edema) further supports this diagnosis, especially with the elevated cardiac filling pressures. *Tension pneumothorax* - A tension pneumothorax would present with **unilateral lung collapse**, **tracheal deviation**, and potentially **hypotension**, none of which are explicitly described or suggested by the CXR. - While it causes respiratory distress, the hemodynamic parameters (CVP and PCWP) and bilateral CXR findings are inconsistent with a pneumothorax. *Transfusion related acute lung injury* - TRALI also causes acute respiratory distress and hypoxemia after transfusion, but it is characterized by **non-cardiogenic pulmonary edema** with a **normal or low CVP** and **normal or mildly elevated PCWP** (<18 mm Hg), differentiating it from TACO. - The elevated CVP and PCWP strongly point away from TRALI, which is immune-mediated and involves capillary leakage. *Mismatched blood transfusion* - An acute hemolytic transfusion reaction (mismatched blood transfusion) typically presents with symptoms such as **fever, chills, flank pain, hemoglobinuria**, and potentially **DIC** or **renal failure**. - While it's a serious transfusion reaction, the primary clinical presentation and elevated cardiac filling pressures are not characteristic of an acute hemolytic reaction.

Q: A 65-year-old man presents with bone pain, fatigue, and hypercalcemia. Bone marrow biopsy reveals 30% plasma cells, and skeletal survey shows lytic lesions. His urinary and serum electrophoresis report is given below. Which of the following is a prognostic marker for this patient?

Beta 2 microglobulin. ***Beta 2 microglobulin*** - The serum/urine electrophoresis shows a **monoclonal gamma spike**, which is characteristic of a **monoclonal gammopathy**, such as **multiple myeloma**. - **Beta 2 microglobulin** is a prognostic marker for multiple myeloma, reflecting **tumor burden** and **renal function**. *Beta 2 transferrin* - **Beta 2 transferrin** is a unique isoform of transferrin found in **cerebrospinal fluid (CSF)** and is used to detect CSF leaks. - It has no known role as a prognostic marker in **monoclonal gammopathies**. *Beta 2 glycoprotein* - **Beta 2 glycoprotein I** is involved in the coagulation cascade and is primarily associated with **antiphospholipid syndrome**. - It is not a prognostic marker for **monoclonal gammopathies**. *Beta 2 microalbumin* - The term **microalbumin** refers to a small amount of albumin in the urine, indicating **early kidney damage**. - While kidney involvement can occur in multiple myeloma, **beta 2 microalbumin** itself is not the primary prognostic marker in this context.

Q: The patient with the following hematological abnormality presents with severe chest pain, difficulty in breathing, fever, hypoxemia, and a new pulmonary infiltrate on chest X-ray. All are useful for managing this emergency except:

Hydroxyurea. ***Hydroxyurea*** - **Hydroxyurea** is a long-term disease-modifying therapy for sickle cell anemia, increasing the production of **fetal hemoglobin (HbF)**, which reduces sickling. - While crucial for preventing future crises and complications, it is not an acute treatment for an emergency like acute chest syndrome, as its effects take weeks to months to manifest. *Packed RBC* - **Simple transfusion with packed red blood cells** can be useful in selected acute chest syndrome cases, particularly when the patient has significant anemia or moderate disease, as it improves oxygen-carrying capacity without the complexity of exchange transfusion. - It is a recognized acute intervention in appropriate clinical scenarios. *Partial exchange transfusion* - **Partial exchange transfusion** is a critical intervention during sickle cell crises with severe complications like acute chest syndrome. - It rapidly reduces the percentage of **sickle hemoglobin (HbS)** and concurrently provides normal red blood cells, improving oxygen-carrying capacity and reducing blood viscosity without significantly increasing total red blood cell mass. *Morphine* - **Morphine** may be used for pain management in sickle cell crisis, including the severe chest pain associated with acute chest syndrome; however, it must be carefully titrated while monitoring for respiratory depression and excessive sedation, as these can worsen hypoxemia in acute chest syndrome. - Adequate pain control helps reduce patient distress, but opioid-induced hypoventilation poses a risk that requires close monitoring.

Q: An infant with HbSS disease presents with the following peripheral smear and X-ray. What will this patient most likely present with:

Hand-foot syndrome. ***Hand-foot syndrome*** - The peripheral smear shows numerous **sickle cells** (elongated, crescent-shaped red blood cells), indicating **sickle cell anemia**. The X-ray depicts **diffuse soft tissue swelling and osseous changes of the small bones of the hands/feet (dactylitis)**, which is a characteristic finding in sickle cell disease due to vaso-occlusive infarcts in the small bones. - **Hand-foot syndrome (dactylitis)** is an initial presentation of sickle cell anemia in infants and young children, characterized by painful swelling of the hands and feet caused by **vaso-occlusion** in the small bones. *Black urine* - **Black urine (alkaptonuria)** is associated with an inborn error of metabolism where homogentisic acid accumulates, leading to ochronosis, not sickle cell disease. - This symptom is not typical for the peripheral smear and X-ray findings presented. *Elevated haptoglobin* - Haptoglobin binds to **free hemoglobin** and is *decreased* in hemolytic anemias like sickle cell disease, as accelerated red blood cell destruction leads to its consumption. - Therefore, an elevated haptoglobin level would argue against **hemolytic anemia**. *Aplastic crisis with pallor* - While aplastic crisis (typically triggered by Parvovirus B19) can occur in sickle cell disease, it presents with sudden drop in hemoglobin and reticulocytopenia rather than the vaso-occlusive findings depicted in the X-ray (dactylitis pattern) and peripheral smear shown here. - Aplastic crisis does not explain the bony changes seen on X-ray in this clinical context.

Question 1

A 16-year-old boy presents with jaundice and splenomegaly. His father had a similar illness during adolescence. MCHC : high? What is the most likely diagnosis?

Accepted Answer

B. Hereditary spherocytosis

Answer

D. AIHA

Answer

C. Thalassemia major

Answer

A. IDA

Question 2

Which of the following is the most common inherited bleeding disorder?

Accepted Answer

Von Willebrand Disease

Answer

Hemophilia

Answer

Factor V Deficiency

Answer

Disseminated Intravascular Coagulation

Question 3

A 40-year-old patient presents with complains of low back pain and development of pallor. During workup very high levels of urinary beta2 microglobulin was noted. X-ray skull was performed. Bone marrow aspiration shows >10% plasma cells and serum electrophoresis shows M protein >30 g/L. All are useful for management of this patient except?

Accepted Answer

Melphalan

Answer

Lenalidomide

Answer

Bortezomib

Answer

Autologous transplantation

Question 4

Comment on the diagnosis in the patient shown below?

Accepted Answer

Hand and foot syndrome

Answer

Squaring of metacarpals

Answer

Sub-periosteal resorption

Answer

Peripheral oedema with iron deficiency anemia

Question 5

An 8-year-old child of migrant construction worker presents with fever for last 2 weeks with progressive pallor. Complete blood counts reveal pancytopenia, malaria work up is negative, Total serum protein is 7 gm% with Serum Globulin of 4.5 g/dL, serum albumin 2.5 g/dL. Bone marrow study shows?

Accepted Answer

L.D bodies

Answer

Aplastic crisis

Answer

Myelofibrosis

Answer

Myeloid metaplasia

Question 6

In a stable, nonbleeding patient with hypoproliferative thrombocytopenia and no additional bleeding risk factors, what is the threshold for prophylactic administration of the following blood product?

Accepted Answer

$<10,000$

Answer

$<5,000$

Answer

$<20,000$

Answer

$<50,000$

Question 7

A patient in ICU was given blood transfusion. 3 hours later SpO2 is reduced to 75% with respiratory difficulty. CVP is 15 cm water and PCWP is 25 mm Hg . CXR is shown below. What is the diagnosis?

Accepted Answer

Transfusion associated circulatory overload

Answer

Tension pneumothorax

Answer

Transfusion related acute lung injury

Answer

Mismatched blood transfusion

Question 8

A 65-year-old man presents with bone pain, fatigue, and hypercalcemia. Bone marrow biopsy reveals 30% plasma cells, and skeletal survey shows lytic lesions. His urinary and serum electrophoresis report is given below. Which of the following is a prognostic marker for this patient?

Accepted Answer

Beta 2 microglobulin

Answer

Beta 2 transferrin

Answer

Beta 2 glycoprotein

Answer

Beta 2 microalbumin

Question 9

The patient with the following hematological abnormality presents with severe chest pain, difficulty in breathing, fever, hypoxemia, and a new pulmonary infiltrate on chest X-ray. All are useful for managing this emergency except:

Accepted Answer

Hydroxyurea

Answer

Partial exchange transfusion

Answer

Morphine

Answer

Packed RBC

Question 10

An infant with HbSS disease presents with the following peripheral smear and X-ray. What will this patient most likely present with:

Accepted Answer

Hand-foot syndrome

Answer

Elevated haptoglobin

Answer

Aplastic crisis with pallor

Answer

Black urine

Hematology — MCQs

Hematology — MCQs

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