Case Study MICR 410 - Hematology Spring, 2011 Case # 6 Monique Quiroz Mike Pehl Andrew Ho.
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Transcript of Case Study MICR 410 - Hematology Spring, 2011 Case # 6 Monique Quiroz Mike Pehl Andrew Ho.
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Case Study Case Study MICR 410 - HematologyMICR 410 - Hematology
Spring, 2011Spring, 2011
Case # 6Case # 6
Monique QuirozMonique Quiroz
Mike PehlMike Pehl
Andrew HoAndrew Ho
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Case SummaryCase SummaryJohn, a 52-year-old man with hyperuricemia, was seen at the clinic for follow-up evaluation 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 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 enlarging. Originally he denied fatigue, fever, and discomfort. He was examined and a CBC was ordered. The results revealed leukocytosis, thrombocytosis, and anemia. CBC was ordered. The results revealed leukocytosis, thrombocytosis, and anemia.
Physical examination revealed a slightly enlarged liver and palpable spleen. Blood counts Physical examination revealed a slightly enlarged liver and palpable spleen. Blood counts showed:showed:
Hb:Hb: 11.6 g/dL11.6 g/dL MCV = 97 fl NormocyticMCV = 97 fl Normocytic
Hct:Hct: 35%35% MCHC = 33 NormochromicMCHC = 33 Normochromic
RBC:RBC: 3.6 x103.6 x101212/L/L LowLow
WBC:WBC: 26.2 x1026.2 x1099/L/L HighHigh
Platlets:Platlets: 853 x10853 x1066/L/L HighHigh
The blood cell differential showed marked anisocytosis, poikilocytosis with many teardrops, The blood cell differential showed marked anisocytosis, poikilocytosis with many teardrops,
and numerous nucleated RBCs. Immature myeloid cells were found and large platelets.and numerous nucleated RBCs. Immature myeloid cells were found and large platelets. During subsequent visits John complained of fatigue, bone pain, abdominal pain and During subsequent visits John complained of fatigue, bone pain, abdominal pain and
discomfort.discomfort.
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Diagnostic TestsDiagnostic Tests
Bone marrow biopsyBone marrow biopsy
--moderate to marked hyperplasia, clusters of platelets, abnormal moderate to marked hyperplasia, clusters of platelets, abnormal
megakaryocyte morphology, and fibrotic marrow spacesmegakaryocyte morphology, and fibrotic marrow spaces
-dry tap-dry tap
Cytogenetic analysisCytogenetic analysis
-trisomy 8-trisomy 8
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Key Information Pointing to Key Information Pointing to DiagnosisDiagnosis
Physical ExaminationPhysical Examination SplenomegalySplenomegaly Bone PainBone Pain Weakness/fatigueWeakness/fatigue
Peripheral blood smearPeripheral blood smear Immature myeloid cellsImmature myeloid cells Large plateletsLarge platelets AnisocytosisAnisocytosis Poikilocytosis with many teardropsPoikilocytosis with many teardrops Nucleated RBCsNucleated RBCs
Bone marrow biopsy
• Dry tap
• Fibrotic marrow spaces
• Marked hyperplasia
• Clusters of platelets
• Abnormal megakaryocyte morphology
Cytogenetic Testing
• Trisomy 8
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What caused the splenomegaly?What caused the splenomegaly?
Extramedullary hematopoiesisExtramedullary hematopoiesis Culling of the teardrop RBC and immature cellsCulling 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.
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The DiagnosisThe Diagnosis
Myelofibrosis with myeloid metaplasia = MMM Myelofibrosis with myeloid metaplasia = MMM Middle agedMiddle aged AnemiaAnemia HepatosplenomegalyHepatosplenomegaly LeukocytosisLeukocytosis ThrombocytosisThrombocytosis Bizarre, functional plateletsBizarre, functional platelets Hypercellular bone marrowHypercellular bone marrow Fibrotic marrow spaces Fibrotic marrow spaces HyperplasiaHyperplasia Clusters of platletsClusters of platlets Abnormal megakaryocytesAbnormal megakaryocytes
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Pathophysiology of Disease MMMPathophysiology of Disease MMM
• 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
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Therapy and Prognosis for Disease Therapy and Prognosis for Disease MMMMMM
Therapy• 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• Can terminate in acute mylocytic leukemia = AML
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Prevention of Disease MMMPrevention of Disease MMM
Disease is idiopathic, so it is difficult to Disease is idiopathic, so it is difficult to determine how to prevent itdetermine how to prevent it
Unfortunately patient shows genetic Unfortunately patient shows genetic predisposition, so only preventative measures predisposition, so only preventative measures would be to avoid known carcinogens and have would be to avoid known carcinogens and have frequent blood analysisfrequent blood analysis
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Take Home MessageTake Home Message The diagnosis is myelofibrosis with myeloid metaplasia The diagnosis is myelofibrosis with myeloid metaplasia
(extramedullary hematopoiesis) (extramedullary hematopoiesis) Typical symptoms are hepatosplenomegaly, anemia, Typical symptoms are hepatosplenomegaly, anemia,
bone pain.bone pain. The cause of the disease is idiopathic. The cause of the disease is idiopathic. Diagnostic tests include bone marrow biopsy and Diagnostic tests include bone marrow biopsy and
cytogenetics.cytogenetics. Treatment is supportive care, immunosuppressive Treatment is supportive care, immunosuppressive
therapy, and eventually a bone marrow transplant. therapy, and eventually a bone marrow transplant. Prognosis is eventual progression to lethal AML.Prognosis is eventual progression to lethal AML. Prevention is nonexistant.Prevention is nonexistant.
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ReferencesReferences
Mayo Clinic : Myelofibrosis Resource. Mayo Clinic : Myelofibrosis Resource. http://www.mayoclinic.org/myelofibrosis/http://www.mayoclinic.org/myelofibrosis/
Harmening, D.M. Harmening, D.M. Clinical Hematology and Clinical Hematology and Fundamentals of HemostasisFundamentals of Hemostasis. 5. 5thth Edition. Edition. Philadelphia, PA. F.A. Davis Company. Philadelphia, PA. F.A. Davis Company. 2009. 2009.
McQueen, N. (2011, May). Lecture 13. McQueen, N. (2011, May). Lecture 13. Neoplastic Disorders of the Bone Marrow. Neoplastic Disorders of the Bone Marrow. Micr 410. CSULA.Micr 410. CSULA.