[CD56-positive acute myeloid leukemia patient with t(16;21)(p11.2;q22) relapsing with isolated pericardial effusion after HLA-haploidentical peripheral blood stem cell transplantation].

The rare chromosomal translocation t(16;21) has been associated with CD56 expression and extramedullary lesions in acute myeloid leukemia (AML). We herein report the first case of the development of isolated pericardial relapse after HLA-haploidentical peripheral blood stem cell transplantation (haplo-PBSCT). A 42-year-old male AML patient with t(16;21)(p11.2;q22) received haplo-PBSCT at partial remission, and he exhibited dyspnea due to massive pericardial effusion 11 months later. The effusion analysis revealed CD56+ leukemic cells, and G-banded karyotyping of the cells demonstrated t(16;21)(p11.2;q22). Salvage chemotherapy was administered, but only a transient improvement of the effusion was achieved. Moreover, the pleural effusion developed without bone marrow relapse.

[Primary breast diffuse large B-cell lymphoma developing subsequent to estramustine therapy for prostate cancer].

An 85-year-old male presented with 1-year history of a right breast mass. Needle biopsy of the mass revealed diffuse proliferation of large lymphoid cells that were positive for CD20, BCL2, BCL6, and MUM1 and negative for CD5, CD10, MYC, and EBER. The patient was diagnosed as having diffuse large B-cell lymphoma, a type of primary breast lymphoma (PBL). Sex hormone imbalance, which causes conditions such as gynecomastia, is associated with PBL development in males. Estramustine is a nitrogen mustard moiety linked to estradiol. For 5 years, the patient underwent estramustine therapy for treating prostate cancer. Our case suggests an important role of estrogen in PBL development.

[Immunodeficiency-associated Burkitt lymphoma developed in a patient receiving a long-term methotrexate therapy for rheumatoid arthritis].

An increased risk of lymphoproliferative disorders (LPD) has been demonstrated in patients treated with methotrexate (MTX) for rheumatoid arthritis (RA). The role of Epstein-Barr virus (EBV) has been discussed in the pathogenesis of immunodeficiency-associated LPDs. We herein present a RA patient, who developed Burkitt lymphoma during MTX treatment. The patient was a 61-year-old Japanese female with a 10-year history of weekly MTX therapy for RA. She presented with a one-month history of submandibular lymph node swelling and fever. Remarkable increases in serum lactate dehydrogenase and blood EBV DNA were observed. Serology for HIV was negative. Biopsy specimens demonstrated diffuse proliferation of medium-sized lymphoid cells. The cells were positive for CD10, CD20 and BCL6, and negative for BCL2, MUM1, terminal deoxynucleotidyl transferase and CD34. The MIB-1 index was almost 100%. EBV in the tumor cells was identified by using EBV-encoded RNA in situ hybridization. A chromosomal translocation t(8;14) was found and further confirmed by fluorescence in situ hybridization. Her condition improved following discontinuation of MTX and initiation of prednisolone. After three cycles of a dose-reduced CHOP-like regimen, chemotherapy was discontinued due to severe complications. However, there has been no sign of recurrence for six years to date without additional intensive chemotherapy.

[Early onset of anti- erythropoietin antibody-mediated pure red cell aplasia after commencement of subcutaneous administration of epoetin-ß].

We report a 73-year-old Japanese man with early onset pure red cell aplasia (PRCA) caused by subcutaneous administration of recombinant epoetin-ß. Two months after the start of epoetin therapy, he developed PRCA. Anti-erythropoietin (EPO) antibody, detected in the patient's serum by enzyme immunoassay and radioimmunoprecipitation method, inhibited EPO-dependent growth of AS-E2 cells in vitro. Treatment with prednisone (1 mg/kg) significantly reduced antibody levels 3 months later. It is important to have an awareness of antibody-mediated PRCA. Our case shows that subcutaneous epoetin administration produces this complication in the early period of therapy.

Post-transplant food anaphylaxis in an adult cord blood transplant recipient (Ms. No. IJHM-D-20-01037R1).

Transplant acquired food allergy (TAFA) is a well-known complication following pediatric liver transplantation, but post-cord blood transplantation (post-CBT) TAFA has rarely been reported. Here, we describe a case of new-onset food anaphylaxis after CBT in an adult patient that demonstrates that post-CBT allergen-challenge is not a risk for long-term allergic sensitization even in adult recipients. The patient was a 39-year-old Japanese man with aggressive NK cell leukemia. He had no previous history of allergies. After receiving CBT, the patient had an unbalanced diet with high preference for bread, bananas, miso-soup, cow's milk, cheese, egg, sesame and buckwheat soba noodles, and experienced repeated diarrhea. Six months later, he developed symptoms such as vomiting, epigastric pain, diarrhea, high fever and hypotension. The condition was initially diagnosed as enterocolitis, but symptoms recurred after consumption of buckwheat. Anaphylaxis induced by buckwheat was confirmed with serum radioallergosorbent tests (RAST), showing allergen-specific IgE for buckwheat (greater than 100 U/mL, Class 6) and egg ovomucoid (Class 4). Nineteen months after a buckwheat and egg-free diet, serum RAST for buckwheat and egg significantly improved. As a result, the patient acquired a tolerance and was able to consume buckwheat and egg without allergic symptoms.

[Intravascular lymphoma confirmed by brain biopsy, quickly treated by chemotherapy: a case report].

A 60-year-old female with intravascular lymphoma (IVL) presented with the complaint of urinary dysfunction and gait disturbance. T2 weighted MR imaging of the thoracic spinal cord showed a hyperintense lesion, and brain MR imaging indicated hyperintense lesions in the deep white matter. Multiple sclerosis was assumed, so steroid pulse therapy was administered. However, her level of consciousness decreased and her paraplegia progressed. Laboratory data showed that anemia and thrombocytopenia had worsened with high serum LDH and soluble IL-2 receptor levels. Biopsy of bone marrow indicated hypercellularity associated with hemophagocytic histiocytes, although no atypical lymphocytes were detected. Brain MR imaging indicated a new subcortical lesion in the left parietal lobe. One and a half months after admission, an open brain biopsy of the left parietal cortex was performed. Histopathological diagnosis was IVL, large B cell type. Immediately, she underwent CHOP therapy containing rituximab (R-CHOP therapy). After chemotherapy, spinal and brain MR images showed no new abnormal lesions. Clinically, it is difficult to make a diagnosis of IVL in life as it has no characteristic symptoms or radiological findings. Therefore, if a patient is suspected of having IVL, a biopsy of different organs, including brain, is necessary for making an early diagnosis and initiating chemotherapy.

Reversible methotrexate-associated lymphoma of the liver in rheumatoid arthritis: a unique case of primary hepatic lymphoma.

Primary hepatic lymphoma (PHL) is an extremely rare disease, frequently associated with viruses such as hepatitis B virus (HBV), hepatitis C virus (HCV), and human immune deficiency virus (HIV). On the other hand, an increased risk of lymphoproliferative disorders (LPD) has been demonstrated in patients treated with immunosuppressive drugs such as methotrexate (MTX) for rheumatoid arthritis (RA). The role of Epstein-Barr virus (EBV) has been discussed in the pathogenesis of the immunodeficiency-associated LPDs. We here describe a RA patient, who developed PHL during RA treatment. The patient was a 64 year-old Japanese male with a 2-year history of RA, who had been treated with MTX at weekly dose of 8-14 mg for 2 years and infliximab (IFX) for 7 months. He presented with a 2 month history of generalized malaise, right hypochondrium pain and fever. Contrast-enhanced computed tomography (CECT) of the abdomen showed multiple irregular and nodular liver masses with a maximum of 13 cm in diameter on the right liver. Biopsy specimens demonstrated CD20-positve diffuse large B-cell lymphoma (DLBCL), but EBV was not identified by EBV-encoded RNA in situ hybridization. Serology for HBV, HCV, human T-cell leukemia virus I (HTLV-I), and HIV was negative. His symptoms disappeared following discontinuation of RA treatment including MTX. A drastic regression of the tumor masses was further obtained without cytotoxic chemotherapy. In addition, although the patient had no past history of liver dysfunction before MTX therapy, persistent elevation of liver enzymes has been observed during MTX treatment. These findings show a causative role of MTX in the development of reversible PHL in the patient.

Case of adult T-cell leukemia/lymphoma with rapid progression of pulmonary areas of ground-glass attenuation and multiple nodules.

We report a case of adult T-cell leukemia/lymphoma (ATL) with rapidly progressive pulmonary areas of ground-glass attenuation (GGA) and nodules resulting from acute transformation of chronic ATL. A 48-year-old Japanese man was admitted for progressive dyspnea. Chest computed tomography showed rapidly progressive bilateral pulmonary areas of GGA and nodules. Flow cytometry of bronchoalveolar lavage fluid and immunohistochemical examination of lung biopsy specimens revealed invasion of ATL cells. Systemic chemotherapy improved the pulmonary findings. Rapidly expanding areas of GGA and nodules are a rare manifestation of pulmonary invasion of ATL that clinicians should nevertheless keep in mind.