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Summary:A 61-year-old man with angioimmunoblastic lymphoma in first complete remission underwent autologous peripheral blood stem cell transplantation. At 1 month post transplant, asymptomatic large granular lymphocytosis developed. The surface marker profile of the cells was CD3 þ CD8 þ CD56ÀCD57 þ . The disease course was chronic and indolent. The patient remains in complete remission from angioimmunoblastic lymphoma more than 6 months post transplant with persistent large granular lymphocytosis (lymphocyte count, 5-15 Â 10 9 /l). Although post transplantation T-cell lymphoproliferative disorders have mostly occurred in allogeneic transplantation recipients and presented as aggressive lymphomas/leukemias, we suggest that chronic indolent T-cell large granular lymphocytic leukemia can occur after autologous stem cell transplantation. Bone Marrow Transplantation (2004) 33, 99-101. doi:10.1038/sj.bmt.1704298 Keywords: T-cell large granular lymphocytic leukemia; post transplantation lymphoproliferative disorders; autologous peripheral blood stem cell transplantation T-cell large granular lymphocytic (T-LGL) leukemia is a lymphoproliferative disease derived from post-thymic immunocompetent T lymphocytes. 1 Large granular cell morphology, CD3 þ CD56ÀCD57 þ immunophenotype and the clonal rearrangement of T-cell receptor genes characterize T-LGL leukemia, which presents clinically with a chronic indolent disease course, complicated by frequent infections secondary to neutropenia.Post transplantation lymphoproliferative disorders (PTLD) are a well-recognized complication of solid organ and allogeneic bone marrow transplantation, and accumulating data have suggested that aggressive immunosuppression is closely associated with an increased risk of PTLD. 2,3 The majority of PTLD are of B-cell origin and are associated with active infection with Epstein-Barr virus (EBV). T-cell PTLD are much less common but, similar to B-cell PTLD, they mostly present as aggressive lymphomas following a rapidly fatal course. 4,5 EBV is infrequently involved in the pathogenesis. Autologous stem cell transplantation (ASCT) is another transplantation procedure. In spite of the profound myelosuppression associated with autografting, this procedure is inherently less immunosuppressive than allogeneic bone marrow transplantation and we were able to find less than 20 cases in the English literature describing PTLD following ASCT. 6-8 Here, we report the first case of chronic T-LGL leukemia following ASCT for angioimmunoblastic lymphoma. Case reportA 61-year-old man was admitted to our hospital in April 1997 because of fever, skin rash, edema, generalized lymphadenopathy and hemolysis. On physical examination, there was a maculo-papular rash on the trunk and extremities, and superficial lymphadenopathy was found in various regions including the neck, axilla and groin. A left pleural effusion and moderate splenomegaly (20 mm on the left midclavicular line) were also noted. The hemoglobin (Hb) was 8.1 g/dl, white blood cell count (WBC) 9.1 Â 10 9 /l...

We herein report a 24-year-old male construction worker with occupational lead poisoning who presented with acute abdomen and normocytic anemia. The levels of urinary delta-aminolevulinic acid and free erythrocyte protoporphyrin were elevated without any increase in the level of urine porphobilinogen. Detection of an elevated blood lead level of 100 μg/dL confirmed a diagnosis of lead poisoning. Chelation therapy with calcium disodium ethylenediaminetetraacetate resulted in prompt improvement of the clinical symptoms and the blood lead level. Clinicians should be aware that lead poisoning caused by occupational exposure can still occur sporadically in construction workers in Japan.

Activation-induced cytidine deaminase (AID ⁄ AICDA) is required for somatic hypermutation and class-switch recombination of the immunoglobulin gene, and for c-myc translocation of germinal center-derived B-cell lymphoma. In the present study, we attempted to clarify the significance of AID associated with c-myc in the progression of follicular lymphoma (FL) using RT-PCR and quantitative real-time PCR. Tissues from the patients with grade 3 FL expressed relatively higher levels of c-myc and AID. The samples taken from a patient with FL who died within 2 years after the start of treatment showed either no or low expression of AID, despite expressing high levels of c-myc. In order to examine the role of AID expression in rapidly progressive FL, the full-length AID transcript was transfected into AID-negative cell lines established from different patients with rapidly progressive FL. This led to the establishment of AID-expressing transfectants with a low proliferation rate and a significantly increased incidence of G 0 ⁄ G 1 arrest compared with controls. Our results indicate that AID may act as a negative regulator of cell survival in FL when sufficient c-myc is expressed. Switch-off or low expression of AID after c-myc amplification may correlate with the clinical outcomes of FL. (Cancer Sci 2012; 103: 415-421) F ollicular lymphoma is the most common type of low-grade lymphoma, accounting for approximately 22% of all nonHodgkin's lymphomas, and shows an indolent clinical course in up to 70% of cases.(1,2) Its cell origin is normal GCB lymphocytes with ongoing SHM of the Ig gene in association with AID ⁄ AICDA.(3-5) FL cells are typically positive for bcl-2 protein. Approximately 75-90% of FL cells have a t(14;18)(q32;q21) translocation, which is the recognized hallmark for diagnosis of FL.(6) Although this translocation is thought to be associated with oncogenic change, it is not sufficient to cause FL, because IgHbcl-2 transgenic mice do not develop lymphomas, and IgH-bcl-2 translocation can be detected in peripheral blood lymphocytes from healthy individuals. (7)(8)(9) Follicular lymphoma can show histological transformation into diffuse aggressive lymphoma during the clinical course in approximately 30% of patients. (10,11) This transformation is usually associated with acceleration of the clinical course. (12) Transformed FLs generally retain the t(14;18) translocation, (13) and it is believed that other genetic abnormalities are necessary in order for this transformation to occur. These secondary genetic events associated with histological transformation include c-myc amplification and translocation, (14,15) bcl-6 translocation, (16) TP53 mutation, (17) P16 gene inactivation,and c-REL amplification. (19) A series of reports has documented dual-translocation lymphoma harboring both bcl-2 and c-myc translocation, and some of the reported cases have a history of preceding FL. (20,21) C-myc translocation occurs in almost all BLs, (22) 5-15% of DLBCLs, and 2-3% of FLs.(23-25) Although rare cases of histologic...

Myxedema coma is a life-threatening endocrine emergency with a high mortality rate resulting from severe insufficiency of thyroid hormones. Intravenous levothyroxine replacement is considered the standard therapy for myxedema coma in many countries. In Japan, however, although there are diagnostic criteria highly suggestive or diagnostic for myxedema coma, no management strategy has been established, despite the availability of levothyroxine. Here we report a 75year-old man with a history of Alzheimer's disease and schizophrenia who developed somnolence and generalized edema. Except for a pulse rate of 60 bpm, his vital signs and blood oxygen level were stable. Thyroid studies showed an elevated serum thyrotropin level of 219.2 μU/mL and a decreased serum free-thyroxine level of 0.15 ng/dL. On this basis he was diagnosed as having hypothyroidism rather than being highly suggestive for myxedema coma. Daily oral levothyroxine 25 μg was initiated and increased to 50 μg 3 days later. Seven days after being started on levothyroxine, the patient suddenly developed impaired consciousness, hypoxemia, hypotension, hypothermia, and hyponatremia. Electrocardiography revealed junctional bradycardia with Osborne J-wave. Myxedema coma was therefore diagnosed. He went into cardiac arrest in the emergency room but was resuscitated. Despite subsequent intravenous administration of hydrocortisone and levothyroxine, as well as intensive supportive care, he eventually died 12 hours after hospital admission. This case illustrates some of the challenges associated with the management of patients with signs highly suggestive/diagnostic of myxedema coma, including the optimal loading dosage and intervention timing of thyroid hormone replacement.