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  #1  
Old 09-29-2011
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Question Hematology Q?

A 3-year-old girl with Down’s syndrome was admitted because of recurrent chest infections associated with anemia and easy bruising. There was mild hepatosplenomegaly in physical examination. Full blood picture revealed pancytopenia with presence of tear-drop-shaped erythrocytes, granulocytic precursor cells and nucleated RBC precursors in variable numbers in peripheral blood smear. Repeated attempts at bone marrow aspiration and trephine biopsy were unsuccessful. What is the most likely diagnosis?

A) Acute lymphoblastic leukemia
B) Acute myeloblastic leukemia
C) Idiopathic myelofibrosis
D) Essential thrombocythemia
E) Endocardial cushion defect
F) Mitral valve stenosis
G) Chronic lymphocytic leukemia
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Old 09-29-2011
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C- Idiopathic Myelofibrosis.
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Old 09-29-2011
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Down syndrome - B) AML M7
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Quote:
Originally Posted by m82_ghasemi View Post
A 3-year-old girl with Down’s syndrome was admitted because of recurrent chest infections associated with anemia and easy bruising. There was mild hepatosplenomegaly in physical examination. Full blood picture revealed pancytopenia with presence of tear-drop-shaped erythrocytes, granulocytic precursor cells and nucleated RBC precursors in variable numbers in peripheral blood smear. Repeated attempts at bone marrow aspiration and trephine biopsy were unsuccessful. What is the most likely diagnosis?

A) Acute lymphoblastic leukemia
B) Acute myeloblastic leukemia
C) Idiopathic myelofibrosis
D) Essential thrombocythemia
E) Endocardial cushion defect
F) Mitral valve stenosis
G) Chronic lymphocytic leukemia
because of the tear drop shaped RBC and i think she would get increase her risk of ALL as she is Down syndrom when she is in her 40s
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C. Idiopathic myelofibrosis
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Old 09-30-2011
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C) Idiopathic myelofibrosis
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I m with c because of the tear drop shaped erythrocytes
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Correct Answer Answer

This is a typical case of idiopathic myelofibrosis:

1) Anemia
2) Splenomegaly
3) Blood smear: Teardrop-shaped erythrocytes; Granulocytic precursor cells; and Nucleated RBCs in variable numbers
4) Proliferation of non-neoplastic fibrous tissue within the bone marrow cavity (myelofibrosis)
5) Eventually, patients have thrombocytopenia
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I'll go with C, because of:

- recurrent chest infections, anemia and easy bruising (pancytopenia)

- mild hepatosplenomegaly

- tear-drop-shaped erythrocytes, granulocytic precursor cells and nucleated RBC precursors in variable numbers in peripheral blood smear

IDIOPATHIC MYELOFIBROSIS: it´s a myeloproliferative disease where the bone marrow is replaced with collagen fibers, which causes blood cells to be unable to grow properly in the BM, progressing to PANCYTOPENIA. Some stem cells are able to migrate to the liver and spleen to produce extramedullary haematopoeisis, which produces HEPATOSPLENOMEGALY.

It is very uncommon in pediatric patients, most cases being secondary to other diseases (such as Down's syndrome)
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