[Rinshō ketsueki] The Japanese journal of clinical hematology
-
A 28 year-old woman in the 26th week of pregnancy was admitted to our hospital on February 6, 1993, because of anemia and thrombocytopenia. On admission, her hemoglobin was 8.2 g/dl, platelet count 6.3 x 10(4)/microliter, and WBC 6,300/microliter with 43% blasts. The bone marrow examination showed hyperplastic bone marrow with 38.8% blasts. ⋯ After DCMP therapy, complete remission was obtained. She has been in complete remission for 32 months and her child is growing healthy after overcoming an underweight condition due to premature birth, respiratory distress syndrome, circulation insufficiency and hyperbilirubinemia. This case suggests that in the event of second trimester pregnant patients with acute leukemia, we should wait for the proper time at which successful delivery can be expected, and then intensified remission induction chemotherapy should be carried out after the delivery.
-
Necrotizing fasciitis is a rare but often fatal soft-tissue infection primarily involving the superficial fascia and fat tissue resulting in extensive undermining of surrounding tissues. Skin is initially spared, but as necrotizing fasciitis spreads, all the soft-tissue components, including the skin, become involved. ⋯ The author reported the occurrence of necrotizing fasciitis in a 69-year-old man with multiple myeloma during the granulocytopenic phase after chemotherapy. The successful treatment of necrotizing fasciitis in the present case relied not only on surgical debridement, but also on G-CSF administration.
-
The mechanism of the cause of hyperleukocytosis induced by differntiation induction therapy of acute promyelocytic leukemia (APL) by all-trans retinoic acid (ATRA) was studies. Of 11 patients treated by ATRA in our hospital, 3 developed hyperleukocytosis. Moreover, 2 out of 4 patients with retinoic acid syndrome (ATRA syndrome) had hyperleukocytosis. ⋯ Moreover, APL cells that differentiated into monocytoid cells possessed the capability of producing endogenous cytokines such as TNF alpha, which might be involved in the development of ATRA syndrome. Compared to cells from patients without hyperleukocytosis they had a stronger TNF alpha producing capability and lower sensitivity to TGF beta, showing hyperdifferentiation in response to ATRA. Depending on the case, those with the same sensitivity to ATRA might show different sensitivities to cytokines.
-
Case Reports
[Mixed warm and cold antibody type autoimmune hemolytic anemia associated with systemic lupus erythematosus].
A 56-year-old woman developed mixed warm and cold antibody type autoimmune hemolytic anemia (mixed AIHA) associated with systemic lupus erythematosus. The patient was admitted to our hospital for acrocyanosis and shortness of breath. High fever and jaundice were observed. ⋯ CA disappeared but the direct antiglobulin test remained positive. Mixed AIHA has been defined as the presence of both warm and cold antibodies. In addition, the presence of symptoms of cold agglutinin disease, or low-titer and high thermal amplitude CA might be necessary for the diagnosis of mixed AIHA.
-
Case Reports
[Carrier detection and prenatal diagnosis for hemophilia A using the inversion analysis of the factor VIII gene].
Hemophilia A is an X-linked hemorrhagic disorder caused by heterogeneous mutations in the factor VIII gene. Recently, it was reported that approximately 50% of the cases of severe hemophilia A may be caused by a common inversion in the factor VIII gene. ⋯ We also showed that the inversion analysis of the factor VIII gene was useful for carrier detection and prenatal diagnosis in a hemophilia A family, which we had not been able to diagnose by the analysis of restriction fragment length polymorphisms (RFLPs) of the factor VIII gene. The detection of inversion mutations in the factor VIII gene using non-RI Southern blotting analysis appears to be very useful for the genetic counseling for severe hemophilia A.