[Philadelphia chromosome (Ph1) positive acute myelomonocytic leukemia with esophageal cancer: a case report].

A 62-year old male was admitted to our hospital because of fever and dysphagia on November 14, 1987. The peripheral leukocyte count was 174,400/microliters with 93% blasts and bone marrow aspiration showed that 90.4% of nucleated cells were blasts positive for both myeloperoxidase and alpha-naphthylbutyrate esterase. Chromosome analysis revealed a karyotype of 45XY, 9q+, 16q+, -20 and 22q-. Esophageal X-ray and endoscopy showed abnormalities. Esophageal biopsy revealed squamous cell carcinoma. He was diagnosed as having Ph1 positive acute myelomonocytic leukemia (AMMoL, M4) and esophageal cancer. He was treated with BHAC-DMP and intermediate-dose ara-C therapy for leukemia and a complete remission was obtained by March 25, 1988. As treatment for esophageal cancer, radiation therapy (total 4,200 cGy) was given and followed by chemotherapy with CDDP and 5-FU. However he died on April 8, 1988. Autopsy findings showed disseminated invasion of esophageal cancer. Ph1 positive AMMoL associated with esophageal cancer is extremely rare.

[Tumor-forming type IgA (kappa) multiple myeloma developed into polyclonal hyper gamma-globulinemia after M-protein loss].

A 77 year-old female admitted with costal and right clavicular tumors and multiple osteolytic lesions. In January 1983, a diagnosis of mature type plasmacytoma was made based on the histopathological examination of the right clavicular tumor. The amounts of serum protein and IgA (kappa) M-protein were 7.5 g/dl and 2.1 g/dl, respectively. A myelogram revealed 21% of mature plasma cells with 31.3 x 10(4) nucleated cells/microliter. Four months later following a chemotherapy started in March 1983, the tumor size became smaller with undetected M-protein by an immunofixation method. Besides, a serum protein analysis showed 24.6% of gamma-globulin and 1,980 mg/dl of IgG. However, in December 1983, the right clavicular and costal tumors regrew. The second biopsy specimen showed diffuse proliferated plasmablastoid cells which reacted only to anti-kappa antibody. By August 1984, the patient had systemic subcutaneous tumors as well as polyclonal IgG up to 3,356 mg/dl suggesting rapid progression of the disease. A myelogram showed 7.4% of mature plasma cells. In December 1984, the patient died of complicated obstructive ileus due to multiple mesenteric tumor. In this study we discussed on the role of M-protein loss and increased of normal globulin level in a tumor-forming type multiple myeloma.

[The role of drugs and lymphocytes in granulocyte-macrophage colony formation in patients with drug induced agranulocytosis].

The in vitro effects of the causative drugs and lymphocytes from the patients with agranulocytosis were tested against granulocyte-macrophage colony formation (CFU-C) of bone marrow cells from normal individuals and the patients in recovery stage. A semisolid culture system was used for CFU-C assay. The drug concentrations were adjusted to the therapeutic levels in sera, and the lymphocytes were obtained from the patient's peripheral blood. Three patients with agranulocytosis and one patient with pancytopenia caused by disopyramide, methimazole, sodium valproate, and Towasaal, respectively, were examined. Each of the four drugs except disopyramide suppressed the CFU-C of normal and patient's bone marrow cells in a dose-dependent manner. When the patient's bone marrow cells were cultured with respective drugs and their own lymphocytes or with the culture supernatant of the drug and lymphocytes, CFU-C suppressions was significantly augmented. Phenacetin, an agent of Towasaal, significantly suppressed CFU-C and also CFU-E. These results indicate that humoral factor(s) produced from patient's lymphocytes by reacting with the drugs may function as an immunological mechanism in the patients with drug-induced agranulocytosis.