Transcript Case Study MICR 410 - Hematology Spring, 2011 Case # 6

Case Study
MICR 410 - Hematology
Spring, 2011
Case # 6
Monique Quiroz
Mike Pehl
Andrew Ho
Case Summary
John, a 52-year-old man with hyperuricemia, was seen at the clinic for follow-up
evaluation for splenomegaly. His palpable spleen, noted 18 months earlier, had been
gradually enlarging. Originally he denied fatigue, fever, and discomfort. He was
examined and a CBC was ordered. The results revealed leukocytosis,
thrombocytosis, and anemia.
Physical examination revealed a slightly enlarged liver and palpable spleen. Blood
counts showed:
Hb:
11.6 g/dL
MCV = 97 fl Normocytic
Hct:
35%
MCHC = 33 Normochromic
RBC:
3.6 x1012/L
Low
WBC:
26.2 x109/L
High
Platlets:
853 x106/L
High
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The blood cell differential showed marked anisocytosis, poikilocytosis with many
teardrops, and numerous nucleated RBCs. Immature myeloid cells were found and
large platelets.
During subsequent visits John complained of fatigue, bone pain, abdominal pain and
discomfort.
Diagnostic Tests
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Bone marrow biopsy
-moderate to marked hyperplasia, clusters
of platelets, abnormal
megakaryocyte morphology, and fibrotic marrow spaces
-dry tap
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Cytogenetic analysis
-trisomy 8
Key Information Pointing to
Diagnosis
Physical Examination
 Splenomegaly
 Bone Pain
 Weakness/fatigue
Bone marrow biopsy
• Dry tap
• Fibrotic marrow spaces
• Marked hyperplasia
• Clusters of platelets
• Abnormal megakaryocyte
morphology
Peripheral blood smear
 Immature myeloid cells
 Large platelets
 Anisocytosis
Cytogenetic Testing
 Poikilocytosis with many teardrops •
Trisomy 8
 Nucleated RBCs
What caused the splenomegaly?
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Extramedullary hematopoiesis
Culling of the teardrop RBC and immature cells
Note: His abdominal pain is most likely a result of both the
extramedullary hematopoiesis occuring in the kidneys, liver, and
spleen, as well as thrombosis of the vasculature associated with the
gastroinstestinal tract.
The Diagnosis
Myelofibrosis with myeloid metaplasia = MMM
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Middle aged
Anemia
Hepatosplenomegaly
Leukocytosis
Thrombocytosis
Bizarre, functional platelets
Hypercellular bone marrow
Fibrotic marrow spaces
Hyperplasia
Clusters of platlets
Abnormal megakaryocytes
Pathophysiology of Disease MMM
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Change in hematopoeitic precursor cells
Increased hematopoesis
• Teardrop RBC as they squeeze out of the bone marrow
Hypercellularity of bone marrow
• Associated bone pain and osteosclerosis
Extramedullary hematopoesis
• Splenomegaly
Fibrosis of bone marrow
Anemia
Therapy and Prognosis for Disease
MMM
Therapy
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Immunosupressive therapy
• Slow down rapidly dividing hematopoetic cells
Surgery for splenomegaly
Blood transfusions
Oral chemotherapy or low dose radiation
• Attack rapidly dividing hematopoetic cells
Bone Marrow Transplant
Prognosis
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Can terminate in acute mylocytic leukemia = AML
Prevention of Disease MMM
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Disease is idiopathic, so it is difficult to
determine how to prevent it
Unfortunately patient shows genetic
predisposition, so only preventative measures
would be to avoid known carcinogens and have
frequent blood analysis
Take Home Message
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The diagnosis is myelofibrosis with myeloid metaplasia
(extramedullary hematopoiesis)
Typical symptoms are hepatosplenomegaly, anemia,
bone pain.
The cause of the disease is idiopathic.
Diagnostic tests include bone marrow biopsy and
cytogenetics.
Treatment is supportive care, immunosuppressive
therapy, and eventually a bone marrow transplant.
Prognosis is eventual progression to lethal AML.
Prevention is nonexistant.
References
Mayo Clinic : Myelofibrosis Resource.
http://www.mayoclinic.org/myelofibrosis/
 Harmening, D.M. Clinical Hematology and
Fundamentals of Hemostasis. 5th Edition.
Philadelphia, PA. F.A. Davis Company.
2009.
 McQueen, N. (2011, May). Lecture 13.
Neoplastic Disorders of the Bone Marrow.
Micr 410. CSULA.
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