A 66-year-old woman presented with anemia in January 2006. She was admitted to our Department in February, after laboratory data showed pancytopenia and bone marrow biopsy reticulin fibrosis. The results of the diagnostic work-up, which included the anti-SS-A antibody, anti-SS-B antibody positivity and salivary gland scintigraphy, Schirmer test and Rose Bengal test, supported the classification criteria of Sjögren syndrome. Due to secondary myelofibrosis accompanied by Sjögren syndrome, she was started on prednisolone (PSL) and recovered completely from the anemia and thrombocytopenia. After the PSL was tapered, a recent follow-up indicated that the peripheral blood had normalized with the PSL therapy. As a causal disease of autoimmune myelofibrosis in collagen disease, systemic lupus erythematosus occurs frequently. This patient is considered to be a rare case in whom secondary myelofibrosis was accompanied by Sjögren syndrome.

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Acquired hemophilia is a life-threatening bleeding disorder by the development of autoantibody against factor VIII. The therapeutic approach relies on steroid, cyclophosphamide and/or cyclosporine. A 64-year-old man was referred to our hospital with extensive hematoma in both psoas muscles, severe anemia of 6.8 g/dl, prolonged activated partial thromboplastin time over 200 seconds, and factor VIII coagulation activity (FVIII: C) of 1.9%. A factor VIII inhibitor was detected at 118 Bethesda units (BU). The diagnosis of acquired hemophilia was made in the absence of a detectable cause. The inhibitor was IgG with a subclass of IgG4 and reacted with 72 kDa fragment of factor VIII light chain. Steroid pulse therapy following steroid treatment resulted in the resolution of acquired hemophila with marked and prolonged efficacy.

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The authors encountered a 7-year-old girl with a huge brain abscess and invasive pulmonary lesion due to fungus, who had been treated for acute myelogenous leukemia (AML). Although she was administered voriconazole to prevent fungal infection, she developed partial seizure and paralysis of the left side because of the huge brain abscess. Fungus culture and serum fungal markers, including Aspergillus antigen, were all negative. She underwent drainage and surgical resection of necrotic tissue after antifungal agents, including liposomal amphotericin B (L-AMB). Resection pathology revealed localized fungal infection, suspected as due to zygomycosis. The cerebral lesion reduced after the operation and the pulmonary lesion also vanished. We discontinued AML treatment because of the severe fungal infection; however, she has remained in continuous remission. Although Lipo-AMPH and itraconazole are comparatively effective for zygomycosis, progressive disseminated zygomycosis is extremely intractable. Our case underlines the feasibility and successful application of combined conventional antifungal agents and surgical resection in such a patient.

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We describe here a case of primary AL amyloidosis associated with IgD monoclonal gammopathy of undetermined significance. A 73-year-old man was referred to our hospital with suspected multiple myeloma due to renal failure and urinary Bence Jones protein. Although serum electrophoresis revealed IgDλ monoclonal protein, the bone marrow did not showed plasma cell proliferation. Systemic bone survey disclosed no lytic bone lesions. Because the patient had macroglossia and multiple ecchymosis in the face and neck, primary amyloidosis was suspected. Skin biopsy revealed extensive deposition of amyloid which was positively stained by Congo red dye. A diagnosis of primary AL amyloidosis associated with IgD monoclonal gammopathy was made. The patient was also complicated renal failure that eventually needed hemodialysis. To our knowledge, this is the first report of primary AL amyloidosis associated with IgD monoclonal gammopathy with undetermined significance.

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Tumor lysis syndrome (TLS) is a severe complication of chemotherapy brought about by the rapid destruction of tumor cells. TLS is usually diagnosed by elevation of intracellular enzymes and no specific abnormality is found in complete blood counts. We present a 22-year-old woman with acute lymphoblastic leukemia (ALL) complicated with TLS, in whom elevation of leukocytes and platelet count was observed due to fragmented leukocytes. The day after initiating chemotherapy, a rapid increase in intracellular enzymes was found and a diagnosis of TLS was made. Her leukocyte and platelet counts increased from 8,400/ml to 42,600/ml. and from 43,000/ml to 231,000/ml, respectively. Many fragmented leukocytes were found in her peripheral blood picture. The automated hematology analyzer counted these fragments as leukocytes or platelets, with resulting pseudo-leukocytosis and pseudo-thrombocytosis. When evaluating laboratory data of TLS, it is necessary to focus on the peripheral blood picture to avoid misunderstanding the blood cell counts.

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A 73-year-old woman was diagnosed with diffuse large B-cell lymphoma of the uterus (Stage IVB). After 3 courses of CHOP therapy, right abducens nerve paralysis appeared and was diagnosed as central nervous system infiltration with lymphoma cells. Although partial remission was obtained by chemotherapy with methotrexate, numbness and muscle weakness of all four limbs appeared asymmetrically and progressed subacutely. Nerve conduction velocity examination revealed mononeuritis multiplex, but we could not reach a final diagnosis. Steroid pulse therapy, chemotherapy including high-dose methotrexate, and radiation therapy were ineffective. On autopsy, histological examination of the peripheral nerves revealed systemic neurolymphomatosis.

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A 55-year-old man with acute promyelocytic leukemia in the first relapse was treated with arsenic trioxide as salvage therapy. After obtaining molecular remission, he underwent autologous peripheral blood stem cell transplantation (PBSCT) with busulfan and melphalan conditioning. The transplant dose of CD 34-positive cells was sufficient, and engraftment was prompt. Platelet count increased to 320×10⁹/l on day 21; however, it rapidly decreased to 27×10⁹/l on day 37. Despite treatment with corticosteroid, the platelet count decreased to 6×10⁹/l on day 55. About one month after cyclosporine administration, thrombocytopenia gradually improved. This clinical course suggests immune-mediated thrombocytopenia following autologous PBSCT.

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