We examined the result of cord blood transplantation (CBT) for acute lymphoblastic leukemia (ALL) in children. Fifty ALL patients underwent stem cell transplantation in our hospital. Among these, 23 patients received related bone marrow transplantation and peripheral blood stem cell transplantation (R-BMT/PBSCT), 17 patients received unrelated bone marrow transplantation (U-BMT), and 10 patients received unrelated cord blood transplantation (U-CBT). The 5-year overall survival rates after R-BMT/PBSCT, U-BMT and U-CBT were 64.6%, 32.3%, and 85.7%, respectively. Event-free survivals after 5 years were 59.6%, 14.7%, and 70.0%, respectively. The relapse rate in the U-CBT group was equal to that in the R-BMT/PBSCT group, and the transplant-related mortality of U-CBT was 0%. Our data show that U-CBT should be the first choice for patients with refractory or relapsed ALL who have no related HLA-matched donor.

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To investigate the actual situation of multiple cancers including hematological malignancies, 266 autopsy cases with one or more hematological malignancies were compiled from autopsy case files between January 1995 and October 2006 in our hospital. The median age at death was 75 years (range 48 to 102 yr). Of 266 cases, 72 (27.1%) had multiple cancers. Of these 72 cases, 62 cases were complicated with non-hematological malignancy, and 10 cases showed duplication of other hematological malignancies. Prostate and colon cancers were frequent as complicating cancers. Seventeen of 256 cases without duplication of other hematological malignancies demonstrated 3 or 4 cancers (6.6%). Of 10 cases showing duplication of other hematological malignancies, 9 cases had NHL. The rate of multiple cancers in elderly patients with hematological malignancy was higher than that of non-hematological cancers.

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A 57-year-old male patient in the first remission of acute erythroid leukemia underwent reduced-intensity umbilical cord blood transplantation. He developed grade III acute graft-versus-host disease (GVHD) on day 29. Although the acute GVHD was resolved with tacrolimus and steroid therapy, weakness developed in the left upper extremity on day 59. Neurological examination demonstrated asymmetric muscular weakness of the extremities with the proximal part of the left upper extremity being markedly affected. Neurophysiological studies suggested that this was due to immune-mediated demyelinating neuropathy. Intravenous immunoglobulin (IVIG) therapy was administered at a dose of 0.4 g/kg/day for 5 days and worsening of clinical symptoms ceased. While the patient developed diarrhea and chronic GVHD of the skin and cytomegalovirus (CMV) antigenemia was repeatedly positive, neurological exacerbation was stabilized. Neurological symptoms did not immediately improve after the second and third dose of IVIG. Approximately 50 days after the third dose of IVIG, neurological symptoms improved with the gradual resolution of diarrhea and CMV reactivation. Although the pathophysiology of polyneuropathies after allo-SCT is not well understood, some reports suggest an association with GVHD or alloreactive T cell expansion following antecedent infection. This case provides valuable information regarding the pathophysiology of peripheral neuropathy following allo-SCT.

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A 70-year-old man was diagnosed as having rheumatoid arthritis (RA) in 2005. He was treated with 1 g salazosulfapyridine (SASP) daily for two years. Hematological investigations conducted since 2005 demonstrated hemoglobin concentrations of 8∼9 g/dl, which then dropped to 4.9 g/dl on November 21, 2007, following which he was admitted to our hospital. Megaloblastic anemia associated with SASP treatment and anemia of chronic disorders were diagnosed on the basis of folate deficiency and bone marrow examination. This report describes a case of megaloblastic anemia, which developed two years after starting SASP and promptly recovered after its withdrawal and treatment with folic acid and prednisolone. The doses of SASP prescribed for RA in Japan are less than those prescribed abroad. Megaloblastic anemia associated with SASP treatment for RA is not usually detected in Japan. Currently, SASP is widely used and one of the key drugs in the treatment of RA. This case suggests that SASP therapy in RA might result in megaloblastic anemia.

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May-Hegglin anomaly (MHA) is a rare autosomal dominant disorder characterized by thrombocytopenia, giant platelets, and unique leukocyte inclusion bodies. The diagnosis of MHA has been made by identifying leukocyte inclusion bodies on May-Giemsa stained blood film, however, it is not always easy to detect these findings. Therefore, patients with MHA are often misdiagnosed and managed as having idiopathic thrombocytopenic purpura. MHA is caused by mutations in the MYH9 gene, which encodes the nonmuscle mysosin heavy chain-A (NMMCH-A). Currently, MHA is definitively diagnosed by immunofluorescence study of leukocyte NMMHC-A localization and MYH9 gene analysis. In this study, we reported two sisters with MHA, who showed consistently decreased platelet counts and giant platelets. However, we could not detect inclusion bodies in their leukocytes. Immunofluorescence analysis of NMMHC-A in leukocytes of both sisters showed abnormal NMMHC-A localization. Furthermore, MYH9 gene analysis of both patients showed heterozygous R116C mutation in exon 26. Based on these findings, the two sisters were diagnosed as having MHA. Immunofluorescence analysis of neutrophil NMMHC-A is useful for diagnosis in patients without leukocyte inclusion bodies who are suspected of having MHA.

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We report a case of B-cell prolymphocytic leukemia (B-PLL) that was treated successfully with splenic irradiation (SI). An 86-year-old man underwent a medical examination for lumbago and general fatigue at another hospital in June 2007. A compressed lumbar fracture and splenomegaly were found using computed tomography (CT). Thereafter, the patient consulted our hospital because of leukocytosis. Peripheral blood showed hemoglobin level 9.8 g/dl and white blood cell count 38.1×10⁹/l with 91% atypical cells. Surface marker analysis demonstrated that atypical cells were positive for CD20, CD22, FMC7, surface IgM, surface IgD and kappa, but were negative for CD5, TdT and lambda. The morphology of these cells was compatible with prolymphocytes with prominent nucleoli and condensed nuclear chromatin. A diagnosis of B-PLL was made. SI (total dose 20 Gy) was chosen for the treatment and a single course of SI was very effective without causing any significant adverse events. This case demonstrates that SI may remain valuable for the treatment of B-PLL in an elderly patient.

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Emergency surgery was performed on a 24-year-old female patient with an unknown type of von Willebrand disease (VWD). The patient suddenly developed lower abdominal pain and was transported to our hospital by ambulance. Based on the diagnosis of ovarian bleeding, emergency surgery was indicated. Ristocetin-cofactor activity (VWF: RCo) was between 7% and 14% by measurement with von Willebrand reagent (Dade Behring, Marburg, Germany). A laparoscopic partial resection of the left ovary was performed under administration of factor VIII/VWF concentrate, Confact F^® (Chemo-Sero-Therapeutic Research Institute, Kumamoto, Japan). During and after surgery, there was no abnormal bleeding. The results obtained by measurement by the reagent were similar to the levels of VWF: RCo obtained by the fixed platelet agglutination method. The reagent was useful for diagnosing and monitoring the VWF: RCo level to control bleeding in VWD.

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A 71-year-old male consulted our hospital complaining of right tonsil enlargement. Tonsillectomy was performed. Histological analysis demonstrated AL amyloid deposits obliterating the tonsillar structures. Foreign body giant cell reaction and morphologically normal plasma cells mainly containing light chain λ were present around the amyloid. Serum immunoglobulin levels were normal and M-proteins were not detected. Urinary test for Bence Jones proteins was negative. Bone marrow aspirates did not demonstrate any signs of multiple myeloma. Amyloidosis of the stomach, colon, heart, and skin was ruled out. We report an extremely rare case of amyloidosis localized in the tonsil and review the literature.

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We describe a 44-year-old man with acute myelogenous leukemia who developed thyrotoxicosis after unrelated cord blood transplantation. He complained of fever, general fatigue, tremor and tachycardia on day 63. On examination of thyroid function, free triiodothyronine (23.67 pg/ml) and free thyroxine (5.71 ng/dl) were increased, and thyroid-stimulating hormone (<0.03 μU/ml) was decreased. Antithyroid receptor antibody, antithyroid peroxidase antibody and antithyroglobulin antibody were all negative. The patient was diagnosed as having thyrotoxicosis. His symptoms improved and thyroid function returned to the normal levels within 2 weeks. Thyrotoxicosis is a rare complication, but we should be aware that it may cause idiopathic fever after stem cell transplantation.

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