A 66-year-old man has experienced fatigue, a 5-kg weight loss, night sweats, and abdominal discomfort for 10 months. On physical examination, he has marked splenomegaly; there is no lymphadenopathy. Laboratory studies show hemoglobin, 10.1 g/dL; hematocrit, 30.5%; MCV, 89 gm3; platelet count, 94,000/mm3; and WBC count, 14,750/mm3 with 55% segmented neutrophils, 9% bands, 20% lymphocytes, 8% monocytes, 4% metamyelocytes, 3% myelocytes, 1% eosinophils, and 2 nucleated RBCs per 100 WBCs. The peripheral blood smear also shows teardrop cells. The serum uric acid level is 12 mg/dL. A bone marrow biopsy specimen shows extensive marrow fibrosis and clusters of atypical megakaryocytes. Which of the following is most likely to account for the enlargement in this patient’s spleen?
A 66-year-old man has experienced fatigue, a 5-kg weight loss, night sweats, and abdominal discomfort for 10 months. On physical examination, he has marked splenomegaly; there is no lymphadenopathy. Laboratory studies show hemoglobin, 10.1 g/dL; hematocrit, 30.5%; MCV, 89 gm3; platelet count, 94,000/mm3; and WBC count, 14,750/mm3 with 55% segmented neutrophils, 9% bands, 20% lymphocytes, 8% monocytes, 4% metamyelocytes, 3% myelocytes, 1% eosinophils, and 2 nucleated RBCs per 100 WBCs. The peripheral blood smear also shows teardrop cells. The serum uric acid level is 12 mg/dL. A bone marrow biopsy specimen shows extensive marrow fibrosis and clusters of atypical megakaryocytes. Which of the following is most likely to account for the enlargement in this patient’s spleen?
💡 Explanation
Okay, let's tackle this question. The patient is a 66-year-old male with symptoms like fatigue, weight loss, night sweats, and abdominal discomfort. He has splenomegaly but no lymphadenopathy. The lab results show anemia, low platelets, and a high WBC count with a specific differential. The peripheral blood smear has teardrop cells, and the bone marrow shows fibrosis and atypical megakaryocytes.
First, I need to figure out the underlying condition. The key findings here are the triad of anemia, thrombocytopenia, and leukocytosis with a left shift, along with teardrop cells and marrow fibrosis. These are classic signs of myelofibrosis. The spleen enlargement in myelofibrosis is due to extramedullary hematopoiesis because the bone marrow is fibrotic and can't function properly. The body tries to compensate by producing blood cells in the spleen, leading to its enlargement.
Looking at the options (even though they're not listed here), the correct answer would be myelofibrosis. The other options might include conditions like CML, lymphoma, or other myeloproliferative disorders. CML usually presents with a high WBC and Philadelphia chromosome, but no marrow fibrosis. Lymphoma would have lymphadenopathy. Essential thrombocythemia has thrombocytosis, not thrombocytopenia. So those can be ruled out.
The clinical pearl here is to remember the triad of myelofibrosis: anemia, thrombocytopenia, and extramedullary hematopoiesis leading to splenomegaly. Also, teardrop cells and marrow fibrosis on biopsy are key diagnostic features.
**Core Concept**
This case illustrates **myelofibrosis**, a myeloproliferative neoplasm characterized by bone marrow fibrosis, extramedullary hematopoiesis, and a leukoerythroblastic peripheral blood smear. The spleen enlarges due to compensatory hematopoiesis and sequestration of abnormal blood cells.
**Why the Correct Answer is Right**
The patient’s **marrow fibrosis and atypical megakaryocytes** (on biopsy) confirm myelofibrosis. Splenomegaly arises from **extramedullary hematopoiesis**, where the spleen compensates for the fibrotic marrow. Teardrop cells (dacrocytes) in peripheral blood are classic due to distorted erythropoiesis in fibrotic marrow. Elevated uric acid reflects increased cell turnover from ineffective hematopoiesis.
**Why Each Wrong Option is Incorrect**
**Option A:** *Chronic myeloid leukemia (CML)* presents with a high WBC, neutrophilic left shift, and Philadelphia chromosome; no marrow fibrosis or teardrop cells.
**Option B:** *Lymphoma* causes lymphadenopathy and marrow infiltration but lacks fibrosis or extramedullary hematopoiesis.
**Option C:** *Essential thrombocythemia* features thrombocytosis, not thrombocytopenia, and no marrow fibrosis.
**Clinical Pearl / High-Yield Fact**
Remember the **"myelofib
✓ Correct Answer: A. Extramedullary hematopoiesis
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