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A 29-year-old woman with muscle phosphofructokinase (PFK) deficiency had exercise intolerance, painful cramps, elevation of muscle enzyme levels in the serum and compensated hemolysis. After the restriction of exercise, the creatine kinase level and indirect bilirubin level decreased, and the reticulocyte count and haptoglobin level were normalized. It is suggested that the hemolysis which was accelerated by exercise was improved by restriction of exercise.

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Plasma Interleukin-6 (IL-6) level was measured in 60 patients with disseminated intravascular coagulation (DIC). Plasma IL-6 level was high in patients with DIC, and was particularly high in patients with multiple organ failure (MOF) or poor prognosis. Plasma IL-6 level correlated positively with C-reactive protein in patients without DIC, but not in those with DIC. The increased plasma IL-6 level observed in DIC patients suggests that activation of the immune system is involved in the progression of DIC and in the pathology of organ failure.

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A 66-year-old female was admitted to our hospital with lumbago. On admission, laboratory examination revealed hemolytic anemia. Direct Coombs' test was positive, and also direct monospecific-Coombs' test by anti-C3d serum was positive. Immunoelectrophoresis showed IgA-lambda type M proteins in serum. Bone marrow aspiration disclosed increased atypical plasma cells. X-ray of skull showed punched-out lesion. From these findings, she was diagnosed as IgA (lambda) myeloma complicating with autoimmune hemolytic anemia. Hemolysis was improved by chemotherapy. It was thought that IgA and C3 were related well to hemolysis in this case.

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Case 1: A 68-year-old male, who was admitted to our hospital because of massive intestinal bleeding, died 1 day after admission. Case 2: An 80-year-old male, who was admitted to our hospital because of a loss of consciousness, died 4 days after admission. In both cases prodromic infection was not detected. At autopsy, phagocytosis of RBC and WBC by histiocytes was shown markedly in the spleen in case 1 and in the bone marrow in case 2. So diagnosis of virus-associated hemophagocytic syndrome was made. It might be related to some underlying immunologic derangement, because both cases were in advanced age.

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Case 1: 75 years old male was admitted to our hospital with anterior chest subcutaneous bleeding. Coagulation study revealed that fibrinogen and alpha 2-PI decreased, and FDP, FPA, B beta 15 approximately 42 and D-dimer increased. Case 2: 78 years old male was admitted to Shingu City Hospital with a left hip subcutaneous hematoma. Coagulation study revealed that fibrinogen, ATIII and alpha 2-PI decreased, and FDP increased. US and CT showed abdominal true aneurysm in both cases. Either severe infection or malignancy was not found. Ticropidine and T-AMCHA were medicated for 8 days in case 1, and for 18 months in case 2. Symptom and coagulation study improved in these cases. Due to some side effects such as appetite loss and liver dysfunction in case 1, and diarrhea in both cases, we changed the therapy to mini-dose heparin therapy. This therapy also proved effective. It is concluded that anti-platelet and anti-fibrinolytic therapy are effective for chronic DIC with abdominal true aneurysm.

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The hemostatic abnormality in 18 patients with adult T cell leukemia (ATL) was studied. Activated partial thromboplastin time (APTT) was slightly prolonged and prekallikrein activity was markedly low in these patients. The leukemic cell homogenate from these patients prolonged the recalcification time (RCT) of normal plasma; homogenates containing more than 3 x 10(3) cells/microliter prolonged it, although a lower cell concentration shortened it. The crude anticoagulant fraction from the gel filtration, with a molecular weight of about 34,000, prolonged RCT. The crude anticoagulant did not affect prothrombin time (PT), thrombin activity or activated X activity at any concentration, but prolonged the contact activation test, inhibited the activation of prekallikrein and prolonged RCT of Fletcher trait, Fitzgerald trait and F XII deficient plasma. These effects of ATL cell homogenate were stronger on platelet poor plasma than on platelet rich plasma. Although ATL cells had low procoagulant activity, increase of leukemic cells made anticoagulant activity predominant, might be the cause of hemostatic abnormality or amplify the bleeding tendency in patients with ATL.

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Twenty-one patients were entered for a clinical study of KM 2210, the benzoate of an estradiol-chlorambucil conjugate, in the treatment of hematopoietic malignancies. These included 4 cases of chronic lymphocytic leukemia (CLL), 5 cases of chronic myelogenous leukemia (CML), 5 cases of malignant lymphoma (ML) and 7 cases of multiple myeloma (MM). Twelve cases had prior chemotherapy. KM 2210 was given orally at a dose of 50-300 mg daily. Of 19 evaluable cases, two cases with CLL achieved complete response and 6 cases including 2 with CLL, 2 with CML and 3 with ML achieved partial response. There were no responders among the cases of MM. The partial and complete response rate was 47%. Toxicity included mild breast or nipple pain (28.6%), genital bleeding (9.5%), appetite loss (9.5%) and gynecomastia (4.8%). These side effects may have been due to increased levels of estrogen. Hematopoietic toxicity was mild and well tolerated. No cardiac, hepatic or renal toxicity was observed in this study. These results suggest that KM 2210 might be an effective candidate for the treatment of hematopoietic malignancies, especially CLL.

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We attempted to examine procoagulant activity (PCA), X activator activity (XAA) and plasminogen activator activity (PlgAA) of various leukemic cell lysates: 17 acute myelocytic leukemias (AML), 4 acute promyelocytic leukemias (APL), 9 acute myelomonocytic leukemias (AMMoL), 7 chronic myelocytic leukemias (CML), 4 CML with blastic crisis, 7 T cell acute lymphocytic leukemias (ALL), 8 adult T cell leukemias (ATL), 8 null cell ALL, 6 B cell lymphocytic leukemias. Among those 70 cases, 4 APL, 4 AMMoL and 5 AML were associated with overt disseminated intravascular coagulation (DIC) and 5 T cell ALL, 7 ATL and 2 null cell ALL were associated with hypofibrinogenemia not adapted for DIC. The sample used was the lysate of 10(7) cells. PCA was measured by recalcification time of normal plasma with the cell lysate, XAA and PlgAA was measured by chromogenic substrate. APL and AML, especially those associated with overt DIC, had high PCA, and lymphocytic leukemia generally had low PCA in comparison with normal controls. Total PCA (PCA multiplied by cell count/microliter) was remarkably increased in DIC and mildly increased in ALL with hypofibrinogenemia. The change in XAA and total XAA (XAA multiplied by cell count/microliter) was not remarkable in any leukemia except for T cell ALL and null cell ALL with hypofibrinogenemia. PlgAA was high in lymphocytic leukemias with hypofibrinogenemia, APL and AMMoL with DIC. Total PlgAA (PlgAA multiplied by cell count/microliter) was high especially in T cell ALL and null cell ALL with hypofibrinogenemia. Thus it is probable that PCA is the most important factor causing DIC in myelogenous leukemia and that PlgAA is the most important factor causing hypofibrinogenemia in lymphocytic leukemia. The measurement of these activities in the leukemic cells is valuable in prediction and prevention of the hemostatic disorder in leukemia.

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