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A 66-year-old male presented with fever and erythema at our hospital, and leukoerythroblastosis, anemia, thrombocytopenia, and multiple low-density lesions in the moderately enlarged spleen were detected. Skin tissue revealed CD8 + T cells with the expression of cytotoxic molecule markers involving fat lobules, and subcutaneous panniculitis T-cell lymphoma (SPTCL) was diagnosed. The bone marrow displayed no infiltration of lymphoid tumor cells, but hyperplasia of granulocytes and megakaryocytes with grade 2 stromal fibrosis. In addition, the bone marrow exhibited diffuse 18 F-fluorodeoxyglucose (FDG) accumulation on FDG positron-emission tomography/computed tomography (FDG-PET/CT). Although chemotherapy improved SPTCL, the patient died from leukocytosis with leukoerythroblastosis. We obtained negative results for the JAK2 V617F mutation, and CD34 + cells were elevated in the bone marrow compared with the levels at initial examination. The final diagnosis was concurrent myelodysplastic syndrome (MDS) with fibrosis and SPTCL. This report highlights that it is essential to consider MDS or other myeloproliferative neoplasms (MPN) as possible complications when malignant lymphoma complicates myelofibrosis in the absence of bone marrow infiltration of lymphoma cells. Perhaps, the assessment of clonal markers of MPN and FDG accumulation patterns in the bone marrow by FDG-PET/CT could enable differentiation.

A 65-year-old man with multiple lymphadenopathy presented to our hospital and was diagnosed with StageIVA blastoid-variant mantle cell lymphoma (MCL), with a Ki-67 index of 93%. Partial response was achieved after four courses of CHASER (cyclophosphamide, cytarabine, dexamethasone, etoposide, and rituximab) chemotherapy, and complete response was achieved after autologous stem cell transplantation (ASCT). Six months after ASCT, the MCL relapsed with occurrence of tumors one on the left upper arm and one in the cerebrum, which were proved to be resistant to the conventional chemotherapy and progressed rapidly. These tumors disappeared with scarring following the local irradiation (45 Gy). However, the unirradiated regions became enlarged. The bulky abdominal lesion was treated with local irradiation (41 Gy) combined with 560 mg of ibrutinib but still resulted in progressive disease 1 month after initiating the ibrutinib treatment. Finally, the patient died 5 months post-relapse. The prognosis of patients with blastoid-variant MCL with high Ki-67 index is extremely poor. Furthermore, the risk of central nervous system (CNS) involvement is very high. Therefore, ibrutinib maintenance therapy post ASCT might be a treatment option to prevent CNS involvement. Further efforts might be needed to improve the outcomes of blastoid-variant MCL with a high Ki-67 index.

Dexmedetomidine (DEX) is a sedative used for monitored anesthesia care (MAC). DEX has been used frequently for MAC because of its less respiratory depressant effect We used DEX in four patients with severe complications who needed surgery under MAC. We started MAC with continuous infusion of 0.5-0.9 μtg - kg(-1) . hr(-1) of DEX, without initial loading dose, combined with regional anesthesia, and gradually either increased or decreased continuous infusion according to Ramsay sedation scale (RSS). The simulated plasma concentrations of DEX were calculated by AnestAs- sistTM PK . PD(-1). All patients were well sedated and operations were completed safely, although simulated plasma concentrations of DEX were low. Remarkable cardiovascular responses and respiratory depression were not observed. Our study indicated that the usage of DEX without initial loading dose combined with regional anesthesia could be an option for patients with severe complications undergoing MAC.

A 58-year-old male was diagnosed as having Philadelphia chromosome-positive acute myeloid leukemia and treated with imatinib combined chemotherapy followed by reduced-intensity cord blood transplantation. After transplantation, the graft was rejected but the Philadelphia-positive leukemia clone was eliminated. Following graft rejection, hematopoietic recovery with del (20q) gradually occurred. These findings suggest that this patient had transformed to acute myeloid leukemia from myelodysplastic syndrome with del (20q) after acquisition of the Philadelphia chromosome.

Primary small intestinal lymphoma (PSIL) is a relatively rare form of non-Hodgkin lymphoma, often complicated by bleeding, obstruction, or perforation of the intestine during the clinical course. The initial diagnosis and management of these complications are often difficult in PSIL, because the small intestine is usually inaccessible in routine endoscopy. Recently, total enteroscopy with a double-balloon method, called double balloon endoscopy (DBE), has been developed for the diagnosis and treatment of small intestinal disorders. We report herein on 4 cases of PSIL (2 diffuse large B-cell lymphomas and 2 follicular lymphomas [FLs]). In these cases, DBE was useful in the diagnosis, decision to perform surgery after assessment of bleeding lesion, and treatment of the intestinal stenosis using enteroscopic balloon dilatation. Combination chemotherapy consisting of anthracycline, cyclophosphamide, vincristine, and prednisolone with rituximab was administered in 3 cases, and all achieved complete remission. One patient with FL of the duodenum was treated with rituximab alone, but with little effect. We conclude that DBE is useful in the management of PSIL. More PSIL cases must be analyzed to establish the optimal management of patients with this form of lymphoma.

A 63-year-old man with idiopathic myelofibrosis, diagnosed on Sept. 2004 and treated with hydroxyurea (HU), was admitted to our hospital on March 2005 because of dyspnea and pleural effusion on the right side. Effusion analysis revealed hemothorax due to extramedullary hematopoiesis or bleeding. We performed a thoracoscopic pleural lung biopsy and pleurodesis with minocycline after surgical evacuation. However, bilateral pleural effusion appeared only 2 weeks later, which was thought to be extramedullary hematopoiesis or acute transformation. We adopted a treatment strategy with low dose whole lung irradiation (4.25 Gy/5 fractions) which was followed by complete disappearance of the bilateral pleural effusion. Our present case suggests that low dose whole lung irradiation may be an effective and safe therapy for intractable pleural effusion due to extramedullary hematopoiesis.

There were 16 cases (11 males and 5 females) of SSPE in Okinawa from 1977 to 1999. The incidence was 0.58 per million population per year for the last 23 years, being higher than in other reports in Japan. Six of the 16 cases contracted measles in 1990. The measles antibody of SSPE cases after 1989 became lower than previously.