In Japan, we encountered a pandemic expansion of novel influenza A(H1N1) in September 2009, but the impact on patients with underlying disease remained unclear. The Tokyo Children Cancer Study Group (TCCSG) established a “novel influenza information-sharing system” to share real time information on how the novel influenza affects pediatric patients with cancer or other hematologic disorders. To facilitate reporting, we limited the items to only the basic data (underlying disease, age, sex), influenza-associated data (diagnostic method, therapy and outcome) and allowed space for free comments. We could share the information promptly, and found that this system worked well. One hundred and fifteen patients were reported between September 2009 and February 2010. Although eight patients needed to be hospitalized, none of the patients died, were admitted to intensive care units or demonstrated sequelae. The novel influenza A(H1N1) did not have a strong impact on pediatric patients with cancer or hematologic disorder at least during the 2009-2010 season.

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In a nationwide survey of ATL in Japan, a total of 910 cases of ATL and 7,164 cases of B-NHL as a control disease, newly diagnosed from January 2006 to December 2007 (2 years), were enrolled from 156 hospitals. Male-female ratios were 1.16 for ATL and 1.22 for B-NHL. Among all ATL cases registered, 59.8% were from an HTLV-1 endemic area in Kyushu, and the ratio of ATL to B-NHL in this area was 1 to 3, while that in a non-endemic area in Tokyo was 1 to 40. Compared to previous nationwide studies, the age of ATL patients shifted toward older ages and the mean age gradually increased from 52.7 years in the first survey (cases before 1980) to 61.1 years in the ninth survey (1996∼1997) and, finally, to 66.0 years in the present study (range: 19 to 94, median: 67). On subtype classification, 46.7% were classified as the acute type, 34.8% the lymphoma type, 10.3% the smoldering type, and 8.2% the chronic type, and the rate of the acute type decreased with an increase in the lymphoma type compared to that in previous studies. An increase in the mean age is explained by the high HTLV-1 prevalence in elderly people over 64 years old in endemic areas (20%), and by the continual development of ATL from this large pool of HTLV-1 carriers. According to mortality statistics from the Ministry of Health, Labor and Welfare in Japan, approximately 1,000 people die annually from ATL, a statistic that has not changed at least for the past decade.

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Leukocytosis, splenomegaly, and an increased vitamin B₁₂ level are characteristic findings of chronic myelogenous leukemia in the chronic phase (CML-CP). Here, we report a patient with CML-CP accompanied by megaloblastic anemia. A 61-year-old man consulted our hospital because of anemia and thrombocytopenia. On physical examination, there were no remarkable findings; there was no hepatosplenomegaly. Laboratory findings were: hemoglobin 6.0 g/dl; MCV 113.6 fl; platelet count 100×10⁹/l; white cell count 8.66×10⁹/l; and LDH 1,236 IU/l. Peripheral blood smear demonstrated hypersegmented neutrophils and megalocytes with emergence of myeloblasts, giant metamyelocytes, and nucleated red cells. Vitamin B₁₂ and folic acid levels were low. Bone marrow examination showed megaloblastic change in the erythroblasts and myeloid hyperplasia. Following vitamin B₁₂ and folic acid administration, anemia and thrombocytopenia rapidly improved; thereafter, marked leukocytosis became evident. Based on the presence of t(9;22)(q34;q11) on cytogenetic study and a positive result for Major bcr/abl fusion gene, a diagnosis of CML-CP was established. This case illustrates that ineffective erythropoiesis results in anemia and thrombocytopenia in CML with vitamin B12 and/or folic acid deficiency.

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Intravascular large B-cell lymphoma (IVLBCL) is a rare form of non-Hodgkin's lymphoma characterized by a proliferation of tumor cells within the lumina of small to medium-sized vessels. Because there are few or no concomitant solid lesions, a diagnosis of IVLBCL usually cannot be established by CT or MR imaging. Herein, we describe a case of IVLBCL involving the uterus, in which ¹⁸F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) was useful for diagnosis. A 47-year-old woman was referred to our hospital because of fever and anemia. Laboratory examination demonstrated anemia and thrombocytopenia. Bone marrow aspiration and biopsy showed hemophagocytosis without involvement of lymphoma cells. Random skin biopsy did not demonstrate lymphoma involvement. FDG-PET/CT imaging showed FDG accumulation in the uterus. MR imaging demonstrated uterine leiomyoma only. Based on these findings, uterine endometrial biopsy was performed and histological diagnosis of IVLBCL involving the uterus was established. She received 6 courses of R-CHOP therapy and high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation. At present, she remains in complete remission after 33 months.

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A 75-year-old man was referred to our hospital for marked neutropenia and anemia. Bone marrow examination showed marked hypoplasia with 45.2% infiltration of CD3+, CD8+, CD16+ and CD57+ granular lymphocytes. Monoclonal rearrangement of T-cell receptor gene was observed by Southern blot analysis. Taking these findings together, T-cell large granular lymphocyte leukemia (T-LGL) with bone marrow failure was diagnosed. The patient was treated with immunosuppressive therapy (IST) consisting of anti-thymocyte globulin and cyclosporine. Although pancytopenia subsided after IST, fever and lymphoadenopathy developed on the 29th day after IST. The presence of Epstein-Barr virus (EBV) in peripheral blood was confirmed using real time PCR (3.5×10⁶ copies/10⁶WBC). Although gancyclovir and foscarnet were started, rapidly progressive hepatomegaly and liver dysfunction developed. The patient died on the 42nd day after IST. Autopsy specimen showed infiltration of abnormal CD20-positive large lymphocytes in the portal area of the liver, white pulp of the spleen, kidneys and adrenal glands. The nuclear EBV-encoded RNA (EBER) stain was positive in the abnormal large lymphocytes and a diagnosis of EBV-associated B-cell lymphoproliferative disorder (EBV-LPD) was made. We should regard the potential risk of EBV-LPD after immunosuppressive therapy for patients with bone marrow failure caused by T-LGL.

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The patient was a 58-year-old man, who presented with headache and myodesopsia. He demonstrated papilledema and hemorrhage in the fundus of the left eye and MRI findings showed localized hypertrophic dura mater. He was diagnosed as having hypertrophic cranial pachymeningitis and treated with antibiotics and prednisolone. However, the patient complained of persistent headache. Therefore, CT scans of the chest and abdomen were obtained. These images demonstrated superficial and intraabdominal lymphadenopathy and a histological diagnosis of mantle cell lymphoma was made on biopsy of an inguinal mass. Specimen obtained at craniotomy also showed the same lymphoma cells diffusely infiltrating the dura mater. Complete remission of the lymphoma including disappearance of hypertrophic dura mater was obtained after 4 courses of rituximab plus hyper-CVAD alternating with high-dose methotrexate and cytarabine, and the neurological manifestation improved thereafter. Subsequently, he underwent autologous peripheral blood stem cell transplantation. Hypertrophic pachymeningitis is a chronic progressive inflammatory disorder with hypertrophic dura mater of brain and spinal cord, caused by diverse illnesses. Mantle cell lymphoma infiltrating the dura mater is extremely rare, and has not been reported previously. In some cases of hematological malignancy with hypertrophic pachymeningitis, dural biopsy is required to differentiate the etiology.

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