Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
A Case of Primary Myelofibrosis Transformed into AMMoL After an Extirpation of a Huge Spleen
Hiroo FURUKAWATadashi OHNAKATakamichi OKADAMichio HIRAITakayuki TAKAHASHIHiromu OKADAKiyoshi AKASAKA
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1986 Volume 27 Issue 3 Pages 349-354

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Abstract

A 53-year-old male was first admitted with a chief complaint of abdominal fullness in September 1981. A diagnosis of primary myelofibrosis was made. WBC increased gradually after admission up to 40,000 and giant platelets were observed. A criterion of a diagnosis of polycythemia vera was satisfied.
Findings of a sternal bone marrow biopsy revealed trpical myelofibrosis and lymph node biopsy an extramedullary hematopoiesis.
Selenectomy was performed in March 1983, because of abdominal fullness aggravated due to a huge spleen. Histologic examinations of the resected spleen, which weighted 6,400 Gm., revealed an extramedullary hematopoiesis.
After splenectomy hepatomegaly progressed and at the end of June a normocellular aspirate of the bone marrow led to a suspicion of AMMoL. In July, abnormal cells appeared in the peripheral blood and a definite diagnosis of AMMoL was made. The size of the platelets grew larger. Low dose Ara-C and Indomethacin treatments were ineffective. Leukemic changes were considered in all the three hematopoietic systems.
He died in October. A diagnosis on autopsy was ileus of the small intestine due to the oppression of a huge liver (4,100 Gm.), AMMoL and myelofibrosis.

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© 1986 The Japanese Society of Clinical Hematology
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