Haematology · NEET PG Preparation Medicine
A 68-year-old man is evaluated for progressive fatigue, early satiety, and massive splenomegaly. Complete blood count shows hemoglobin of 9.2 g/dL, l…
Practice question from Haematology (NEET PG Preparation Medicine), with the correct answer and a step-by-step solution.
hard · MCQ · Q-87B024
A 68-year-old man is evaluated for progressive fatigue, early satiety, and massive splenomegaly. Complete blood count shows hemoglobin of 9.2 g/dL, leukocyte count of 38,000/uL with a leukoerythroblastic blood picture (teardrop cells, nucleated red blood cells, myelocytes, and metamyelocytes), and platelet count of 450,000/uL. Bone marrow aspiration results in a dry tap. Trephine biopsy reveals marked fibrosis and atypical megakaryocytic hyperplasia. Cytogenetic analysis reveals a JAK2 V617F mutation. What is the definitive curative treatment for this condition?
- A.Allogeneic hematopoietic stem cell transplantationCorrect answer
- B.Ruxolitinib monotherapy
- C.Hydroxyurea combined with splenectomy
- D.Serial therapeutic phlebotomies
Solution
The clinical presentation (massive splenomegaly, leukoerythroblastic blood smear, dry tap, and bone marrow fibrosis with JAK2 mutation) is diagnostic of primary myelofibrosis (PMF). The only potentially curative treatment for primary myelofibrosis is allogeneic hematopoietic stem cell transplantation (HSCT), typically reserved for patients with intermediate-2 or high-risk disease. Ruxolitinib (JAK inhibitor) and hydroxyurea are palliative options used for symptom control and splenomegaly.
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