1988 Volume 29 Issue 5 Pages 738-742
A-71-year old man was admitted to Kitano Hospital in December 1985 because of palpitation, shortness of breath and leukocytosis. The peripheral blood showed RBC 173×104/μl, Hb 5.4g/dl, PLT 23×103/μl, and WBC 49,600/μl with neutrophilia. The bone marrow smear and biopsy showed a myeloid hyperplasia, and NAP score was consistently high. The serum levels of LDH, uric acid, and ferritin were elevated, and that of vitamin B12 markedly high. Both serum and urine lysozyme levels were significantly high. The spleen was moderately enlarged, but no apparent signs of infection or other malignancy were found out. The chromosome analysis of bone marrow cells showed an abnormal karyotype of 7q-, and Ph1 chromosome was not found.
Clonogenic culture study revealed the formation of endogenous CFU-GM colonies. In addition, CFU-GM colonies formed in the standard culture condition failed to respond to PGE2 or neutrophils.
Although administration of dibromomannitol was partially effective, he died of subdural and gastrointestinal bleeding. A postmortem histology of the liver showed the infiltration of granulocytes of each maturation stage into both the portal area and liver sinusoids.
This case appeared unique in the meaning of both cytogenetic and clonogenic culture studies.
