Hematopoietic Stem Cell Transplantation — MCQs
Which of the following is the most common myeloproliferative disorder?
A child undergoes prophylactic irradiation as preparation for bone marrow transplantation (BMT) for treatment of acute lymphoblastic leukemia (ALL). Which of the following cell types will be least affected by the radiation?
A 44-year-old female presented to OPD with complaints of pallor, fatigue, weakness, palpitations and dyspnea on exeion. Blood tests were conducted, which revealed, Anemia Thrombocytopenia Leukocytosis with neutropenia and increased blasts in the peripheral blood smear. Peripheral blood smear The patient was diagnosed with leukemia and she underwent allogenic stem cell transplantation for the same. After 24 days, she again presented with hypotension, tachycardia, and spO2 of 88% along with a new rash from which biopsy was taken and silver staining was done. Lab findings revealed severe Neutropenia. Which is the most likely organism causing the above skin condition: -
Autoimmune hemolytic anemia is seen in: A) Sickle cell anemia B) Chronic lymphocytic leukemia (CLL) C) Acute myelocytic leukemia (AML) D) Multiple myeloma
What is the most effective method to prevent transfusion-associated graft-versus-host disease?
What is the most effective management strategy for hemarthrosis?
An autograft of a burn victim is best described by which one of the following?
Most common hematological malignancy associated with Rheumatoid Arthritis (RA)?
What is a known complication of Parvovirus B19 infection?
Chronic hemolytic anaemia is associated with which of the following -
Hematopoietic Stem Cell Transplantation Indian Medical PG Practice Questions and MCQs
Question 1: Which of the following is the most common myeloproliferative disorder?
- A. Essential Thrombocythemia (Correct Answer)
- B. Polycythemia rubra vera
- C. CML
- D. Myelofibrosis
Explanation: ***Essential Thrombocythemia*** - **Essential thrombocythemia (ET)** is the **most common myeloproliferative neoplasm**, with an incidence of approximately 1.5-2.4 per 100,000 per year. - It is characterized by **persistent thrombocytosis** (platelet count >450,000/μL) and megakaryocytic proliferation [1]. - Commonly associated with **JAK2 V617F mutation** (~55-60%), **CALR mutations** (~25-30%), and **MPL mutations** (~3-5%) [2]. *Polycythemia rubra vera* - **Polycythemia vera (PV)** is the **second most common** classic MPN, with an incidence of approximately 0.8-2.3 per 100,000 per year. - Characterized by increased red blood cell mass, often with leukocytosis and thrombocytosis [1]. - Strongly associated with **JAK2 V617F mutation** (present in >95% of cases) [2][3]. *CML* - **Chronic myeloid leukemia (CML)** has similar incidence to PV (approximately 1-2 per 100,000 per year). - Defined by the presence of the **Philadelphia chromosome (BCR-ABL1 fusion gene)** [2]. - Treated distinctly with tyrosine kinase inhibitors (TKIs). *Myelofibrosis* - **Primary myelofibrosis (PMF)** is the **least common** of the classic MPNs, with an incidence of approximately 0.3-1.5 per 100,000 per year. - Characterized by bone marrow fibrosis, extramedullary hematopoiesis, and splenomegaly [3]. - Associated with **JAK2, CALR, or MPL mutations** [2]. **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. 627-628. [2] 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, p. 624. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 614-615.
Question 2: A child undergoes prophylactic irradiation as preparation for bone marrow transplantation (BMT) for treatment of acute lymphoblastic leukemia (ALL). Which of the following cell types will be least affected by the radiation?
- A. Spermatogonia
- B. Bone marrow
- C. Intestinal epithelial cells
- D. Neurons (Correct Answer)
Explanation: ***Neurons*** - **Neurons** are highly differentiated cells with very low rates of cell division in adults. As radiation primarily targets rapidly dividing cells [4], **neurons are least susceptible** to radiation damage. - While high doses of radiation can eventually damage neurons, their **radioresistance** is significantly higher compared to rapidly proliferating tissues. *Spermatogonia* - **Spermatogonia** are germ cells that undergo continuous and rapid division to produce sperm, making them **highly sensitive to radiation** [2]. - Radiation exposure can lead to **sterility** due to the destruction of these rapidly dividing cells [2]. *Bone marrow* - The **bone marrow** contains hematopoietic stem cells that are responsible for the continuous production of blood cells, involving **rapid cell division** [3]. - It is one of the most **radiosensitive tissues** [1], and radiation exposure can lead to **myelosuppression** and pancytopenia. *Intestinal epithelial cells* - **Intestinal epithelial cells** have a high turnover rate due to their constant shedding and replacement [5], making them **very sensitive to radiation** [1]. - Radiation damage to these cells can cause **mucositis, nausea, vomiting, and diarrhea**. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. (Basic Pathology) introduces the student to key general principles of pathology, both as a medical science and as a clinical activity with a vital role in patient care. Part 2 (Disease Mechanisms) provides fundamental knowledge about the cellular and molecular processes involved in diseases, providing the rationale for their treatment. Part 3 (Systematic Pathology) deals in detail with specific diseases, with emphasis on the clinically important aspects., pp. 112-113. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. (Basic Pathology) introduces the student to key general principles of pathology, both as a medical science and as a clinical activity with a vital role in patient care. Part 2 (Disease Mechanisms) provides fundamental knowledge about the cellular and molecular processes involved in diseases, providing the rationale for their treatment. Part 3 (Systematic Pathology) deals in detail with specific diseases, with emphasis on the clinically important aspects., pp. 113-114. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Inflammation and Repair, pp. 112-113. [4] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Central Nervous System Synapse, pp. 436-437. [5] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. (Basic Pathology) introduces the student to key general principles of pathology, both as a medical science and as a clinical activity with a vital role in patient care. Part 2 (Disease Mechanisms) provides fundamental knowledge about the cellular and molecular processes involved in diseases, providing the rationale for their treatment. Part 3 (Systematic Pathology) deals in detail with specific diseases, with emphasis on the clinically important aspects., pp. 79-80.
Question 3: A 44-year-old female presented to OPD with complaints of pallor, fatigue, weakness, palpitations and dyspnea on exeion. Blood tests were conducted, which revealed, Anemia Thrombocytopenia Leukocytosis with neutropenia and increased blasts in the peripheral blood smear. Peripheral blood smear The patient was diagnosed with leukemia and she underwent allogenic stem cell transplantation for the same. After 24 days, she again presented with hypotension, tachycardia, and spO2 of 88% along with a new rash from which biopsy was taken and silver staining was done. Lab findings revealed severe Neutropenia. Which is the most likely organism causing the above skin condition: -
- A. Neisseria meningitidis
- B. Pseudomonas (Correct Answer)
- C. Staphylococcus aureus
- D. Vibrio vulnificus
Explanation: ***Pseudomonas*** - The clinical presentation of **neutropenia**, fever, and a rapidly progressive skin rash after stem cell transplantation is highly suggestive of **ecthyma gangrenosum**, a severe cutaneous infection typically caused by *Pseudomonas aeruginosa*. - **Silver staining** in a biopsy would highlight bacteria, and *Pseudomonas* is a common cause of severe infections in immunocompromised patients, especially those with **neutropenia**. *Neisseria meningitidis* - While *Neisseria meningitidis* can cause rash (e.g., **petechial or purpuric rash** in meningitis), it is less likely to present as a focal, rapidly necrotic skin lesion like ecthyma gangrenosum, especially in the context of profound neutropenia post-transplant without overt signs of meningococcal disease. - The rash associated with meningococcemia is typically due to **vasculitis and thrombosis**, not direct bacterial colonization leading to necrotic lesions in the same way *Pseudomonas* does. *Staphylococcus aureus* - *Staphylococcus aureus* can cause various skin infections, including **cellulitis, abscesses, or impetigo**, but ecthyma gangrenosum is not its typical presentation. - While *S. aureus* is a significant pathogen in immunocompromised patients, the constellation of severe neutropenia and a rapidly progressive, necrotic skin lesion characteristic of ecthyma gangrenosum points more strongly to *Pseudomonas*. *Vibrio vulnificus* - *Vibrio vulnificus* causes severe skin infections, particularly in individuals with **liver disease** or those exposed to **contaminated seawater** or raw seafood. - This patient's history of leukemia and stem cell transplantation, without mention of relevant exposures, makes *Vibrio vulnificus* a less likely pathogen in this scenario.
Question 4: Autoimmune hemolytic anemia is seen in: A) Sickle cell anemia B) Chronic lymphocytic leukemia (CLL) C) Acute myelocytic leukemia (AML) D) Multiple myeloma
- A. Acute myelocytic leukemia (AML)
- B. Multiple myeloma
- C. Sickle cell anemia
- D. Chronic lymphocytic leukemia (CLL) (Correct Answer)
Explanation: ***Chronic lymphocytic leukemia (CLL)*** - **CLL** is strongly associated with **autoimmune hemolytic anemia** due to immune system dysregulation that leads to production of **autoantibodies** against red blood cells [1]. - The malignant B-lymphocytes in CLL cause **immunologic dysfunction**, making AIHA one of the most common autoimmune complications of this disease [1]. *Sickle cell anemia* - **Sickle cell anemia** causes hemolysis through **intrinsic red blood cell defects** due to abnormal hemoglobin S structure, not autoimmune mechanisms. - The hemolysis is **mechanical** and occurs when sickled cells become rigid and fragile, rather than being mediated by autoantibodies. *Acute myelocytic leukemia (AML)* - **AML** is a rapidly progressing cancer of the myeloid blood cell line, primarily affecting early progenitor cells. - Autoimmune hemolytic anemia is **rarely associated** with AML; the primary hematologic issue is **pancytopenia** due to bone marrow suppression. *Multiple myeloma* - **Multiple myeloma** is a plasma cell dyscrasia characterized by proliferation of malignant plasma cells and production of **monoclonal proteins** [2]. - While other hematologic abnormalities can occur, **AIHA is not a common complication** of multiple myeloma [2].
Question 5: What is the most effective method to prevent transfusion-associated graft-versus-host disease?
- A. Irradiation (Correct Answer)
- B. Washing
- C. Chemical treatment
- D. None of the options
Explanation: Irradiation - **Irradiation** of blood products inactivates donor T-lymphocytes, which are responsible for initiating the immune attack in **transfusion-associated graft-versus-host disease (TA-GVHD)**. - This method is highly effective in preventing TA-GVHD, especially in immunocompromised patients or those receiving blood from biologically related donors. *Washing* - **Washing** removes plasma proteins, antibodies, and some leukocytes but is not effective in removing or inactivating all viable T-lymphocytes that can cause TA-GVHD. - It's primarily used to prevent allergic reactions or to remove excess potassium in certain patient populations. *Chemical treatment* - While experimental chemical treatments for pathogen inactivation exist, there is no widely accepted or clinically proven chemical treatment specifically for preventing **TA-GVHD** by inactivating lymphocytes. - Such methods are not part of routine blood banking practices for this purpose. *None of the options* - This option is incorrect because **irradiation** is a recognized and effective method for preventing **transfusion-associated graft-versus-host disease**.
Question 6: What is the most effective management strategy for hemarthrosis?
- A. Immobilization with a P.O.P. cast
- B. Application of a compression bandage
- C. Needle aspiration to remove excess blood (Correct Answer)
- D. All of the options
Explanation: ***Needle aspiration to remove excess blood*** - **Aspirating the blood** from the joint effectively reduces intra-articular pressure, pain, and inflammation. - This procedure also helps prevent **synovial hypertrophy** and **cartilage damage** caused by the presence of blood in the joint. *Application of a compression bandage* - While helpful for reducing swelling and providing support, a **compression bandage alone** does not remove the accumulated blood. - It may alleviate some discomfort but does not address the underlying issue of **intra-articular blood accumulation**. *Immobilization with a P.O.P. cast* - **Immobilization** can help rest the joint and reduce pain, but it does not remove the blood from the joint space. - Prolonged immobilization can lead to **joint stiffness** and **muscle atrophy**, which are undesirable outcomes. *All of the options* - While compression and immobilization can be supportive measures, they are not the **most effective primary strategy** for managing hemarthrosis. - The direct removal of blood via **aspiration** is crucial for alleviating pressure and preventing long-term joint damage.
Question 7: An autograft of a burn victim is best described by which one of the following?
- A. Transplant from one person to another person
- B. Transplant from one person to a genetically identical person
- C. Transplant from one species to another species
- D. Transplant from one region of a person to another region (Correct Answer)
Explanation: ***Transplant from one region of a person to another region*** - An **autograft** involves transferring tissue from one site to another within the **same individual**. - This type of graft is immunologically optimal as it avoids **immune rejection**. *Transplant from one person to another person* - This describes an **allograft**, where tissue is transplanted between genetically distinct individuals of the **same species**. - **Allografts** carry a risk of rejection and require immunosuppression. *Transplant from one person to a genetically identical person* - This is an **isograft** (or syngraft), occurring between **monozygotic twins** or highly inbred animals. - While genetically identical, it is a specific type of **allograft** and not an autograft. *Transplant from one species to another species* - This describes a **xenograft**, where tissue is transferred between individuals of **different species**. - **Xenografts** face significant immune rejection and ethical considerations, making them less common in standard practice.
Question 8: Most common hematological malignancy associated with Rheumatoid Arthritis (RA)?
- A. Diffuse large B cell lymphoma
- B. Chronic lymphocytic leukemia
- C. T-cell prolymphocytic leukemia
- D. Large granular lymphocytic leukemia (LGLL) (Correct Answer)
Explanation: ***Large granular lymphocytic leukemia (LGLL)*** - **LGLL** is the most common hematological malignancy strongly associated with **rheumatoid arthritis (RA)**, often presenting with features such as **neutropenia** and splenomegaly. - Approximately 80% of patients with LGLL have a **T-cell phenotype**, and a significant subset experiences **autoimmune diseases**, with RA being the most frequent. *Diffuse large B cell lymphoma* - While patients with **RA** have an increased risk of **lymphoma**, **diffuse large B-cell lymphoma (DLBCL)** is a more aggressive type but not the most common hematologic malignancy directly associated with the disease itself in terms of prevalence [3]. - Inflammatory conditions like **RA** can contribute to chronic immune stimulation, increasing the risk of certain lymphomas, but LGLL holds a more direct and prevalent association [1]. *Chronic lymphocytic leukemia* - **Chronic lymphocytic leukemia (CLL)** is a lymphoproliferative disorder of **B lymphocytes**, but it does not have a particularly strong or common association with **RA** compared to LGLL [2]. - The elevated risk of hematological malignancies in RA patients typically points more towards lymphoproliferative disorders driven by specific immune dysregulations characteristic of RA. *T-cell prolymphocytic leukemia* - **T-cell prolymphocytic leukemia (T-PLL)** is a rare and aggressive **T-cell leukemia** that generally presents with a high white blood cell count and splenomegaly, but it is not commonly linked with **RA**. - Its clinical presentation and biology are distinct from the more indolent leukemias like LGLL that are often seen in conjunction with autoimmune conditions.
Question 9: What is a known complication of Parvovirus B19 infection?
- A. Erythema infectiosum
- B. Arthritis
- C. Aplastic anemia (Correct Answer)
- D. All of the options
Explanation: Aplastic anemia - Parvovirus B19 has a tropism for erythroid progenitor cells in the bone marrow, specifically targeting and destroying them [1]. - This destruction can lead to a transient aplastic crisis, especially in individuals with pre-existing hemolytic conditions, causing a severe drop in red blood cell production [1]. Erythema infectiosum - This is the most common clinical manifestation of Parvovirus B19 infection, also known as fifth disease, characterized by a "slapped cheek" rash [1]. - While it is a symptom or disease caused by the virus, it is not considered a complication in the sense of a secondary, adverse outcome. Arthritis - Arthralgia and arthritis are common manifestations of Parvovirus B19 infection, particularly in adults, especially women [1]. - Similar to erythema infectiosum, it is a direct clinical manifestation rather than a "complication" representing a secondary, undesirable event. All of the options - While erythema infectiosum and arthritis are common clinical presentations of Parvovirus B19, they are direct disease manifestations. - Aplastic anemia stands out as a true complication, representing a secondary and potentially severe adverse outcome due to the virus's specific cellular tropism [1].
Question 10: Chronic hemolytic anaemia is associated with which of the following -
- A. Brown Pigment stone of the gall bladder
- B. Black Pigment stone of the gall bladder (Correct Answer)
- C. Uric acid Renal Calculus
- D. Intestinal Obstruction
Explanation: ***Black Pigment stone of the gall bladder*** - Chronic hemolytic anemia leads to increased **bilirubin**, particularly unconjugated bilirubin, which can result in the formation of **black pigment stones** [1]. - These stones are associated with conditions causing **excess bilirubin production**, such as sickle cell disease and thalassemia. *Brown Pigment stone of the gall bladder* - Brown pigment stones are primarily associated with **infection** and **biliary tract disorders**, not directly with chronic hemolytic anemia. - They are mainly composed of **calcium bilirubinate**, which arises in cases of **bacterial infections** or parasitic infestations. *Intestinal Obstruction* - While hemolytic anemia can have various complications, it is **not directly linked with intestinal obstruction**. - Obstruction typically arises from **mechanical causes** such as adhesions, tumors, or hernias, rather than from hemolytic processes. *Uric acid Renal Calculus* - Uric acid stones are formed due to conditions leading to **hyperuricemia** and are not a primary consequence of chronic hemolytic anemia. - They are often associated with **gout** and **certain metabolic disorders**, rather than hemolytic processes.
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