慢性好中球性白血病を発症した第Ｖ，第ＶＩＩＩ因子合併欠乏症

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  • Combined Factors V and VIII Deficiency associated with Chronic Neutrophilic Leukemia

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Combined factors V and VIII deficiency, a rare hereditary coagulopathy, was found in a 64-year-old woman suffering from a chronic neutrophilic leukemia (CNL), also a rare myeloproliferative disease. Despite of a marked splenomegaly and mature neutrophilia both in the peripheral and bone marrow blood, neither Ph1 chromosome nor BCR/ABL gene arrangement was found to be present. These findings allowed us to make a diagnosis of CNL. A cytogenetic study showed an abnormal karyotype of del (11) (q23). The observed hemorrhagic diathesis and the elongated prothrombin and partial thromboplastin times may well be attributed to a combined factos V and VIII deficiency, i. e., 22% and 45% of normal in activity, and 10% and 51% of normal in antigen for respective factors in plasma.<br>None of the circulating anticoagulants, the factor VIII inhibitor, the lupus anticogulant or an anti-cardiolipin antibody was detectable in her plasma. The coagulant activities of factors V and VIII and the peripheral leukocyte counts were not correlated with each other all through her clinical course. Thus, the factors V and VIII deficiency may not have resulted from CNL. On the basis of family studies, the combined factors V and VIII deficiency is unlikely to be an incidental combination of independent deficiencies of factors V and VIII.<br>Thus, this paper seems to deal with a new type of hitherto unreported association of a combined factors V and VIII deficiency and CNL.

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