A 49-year-old woman with severe thrombocytopenia was admitted after an episode of syncope. She also had anemia, fever, pleural effusion and ascites, and multiple lymphadenopathies subsequently appeared. Her bone marrow showed increased megakaryocytes with mild fibrosis, whereas her lymph nodes lacked histologically specific findings. Her presentation was not consistent with multicentric Castleman's disease, angioimmunoblastic T-cell lymphoma, systemic lupus erhythematosus or any other well-recognized entities. Her clinical features were, however, thought to be compatible with TAFRO (thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly) syndrome. Corticosteroid therapy induced a partial remission of fever and systemic fluid retention, but thrombocytopenia persisted. After additional immunosuppressive therapy with cyclosporin A, her symptoms showed full resolution. 〔J Clin Exp Hematop 53(1) : 95-99, 2013〕

Primary renal lymphoma (PRL) is a rare disease which is often mistaken for renal cell carcinoma. In the present study, a 56-year-old man visited a clinic complaining of an intermittent fever and right flank pain. A computerized tomography examination revealed a hypoenhancing mass in his right kidney. Radical nephrectomy was conducted, and a diagnosis of diffuse large B-cell lymphoma was confirmed. The present case was determined to be a true PRL according to the results of a positron emission tomography examination and a bone marrow biopsy to rule out any lymphoma invasions apart from the right kidney.

Hemophagocytic lymphohistocytosis (HLH) is commonly associated with infectious diseases or T/NK celllymphoma; however that with Hodgkin lymphoma (HL) was rarely reported. Herein, we describe a young male diagnosed with lymphocyte-depleted HL (LD-HL) complicated by HLH as an initial manifestation. He was given high-dose steroid therapy plus recombinant thrombomodulin, and subsequent ABVd (doxorubicin, bleomycin, vinblastine, dacarbazine) treatment. In spite of the achievement of a partial remission treated with one cycle of ABVd, he relapsed after 3 cycles. To our knowledge, the present case is very rare, and more intensive treatment might be needed for the long-term control of HLH-complicated HL.

Nilotinib, a second-generation tyrosine kinase inhibitor with 20- to 30-fold greater potency than imatinib, was developed to overcome imatinib intolerance or resistance. Recently, nilotinib has been approved as a first-line treatment for chronic myelogenous leukemia in the US and Japan. Tumor lysis syndrome (TLS) is an extremely rare adverse event that can occur during treatment with nilotinib, with only a few reported cases to date. Herein, we report two patients who developed TLS soon after the start of treatment with nilotinib. While in the first case, which co-presented with underlying mild-to-moderate renal insufficiency due to polycystic kidney disease, the TLS resolved on discontinuation of the drug, the second patient, who had an exceedingly high white blood cell count, presented with disseminated intravascular coagulation and severe liver injury triggered by TLS that developed after the start of nilotinib treatment, and died of multiple organ failure. Therefore, caution is necessary when this drug is used in the first-line setting in patients with renal insufficiency or a high tumor burden.

Acute myeloid Leukemia(AML) is known as heterogeneous disease, especially in adult AMLs. FLT3/ITD and NPM1 mutation are frequentry seen at the same time, and expected as a role of stratification factor. FLT3/ITDs are the most frequent genetic alterations in AML, found in approximately 20% of adult AMLs. FLT3/ITD is associated with leukocytosis and worse prognoses. ITD mutations vary in size and location, ranging in size from 3 to hundreds of nucleotides. Recently, it is reported that nucleophosmin gene(NPM1) is mutated in a high proportion of gene(NPM1) is mutated in a high proportion of adults with AML and associated with a normal karyotype and FLT3 mutations. In this study, we examined prognostic significance of FLT3/ITD and NPM1 mutation in de novo adult AMLs. We analyzed 51 AML patients(median age:48y/o, range:17–83y/o). Mainly almost patients underwent JALSG AML97 protocol therapy. Acute promyelocytic leukemia was also comprised in this analysis, and 18 of 54 cases were underwent bone marrow transplantation(BMT). Genomic DNA was amplified by means of a polymerase chain reaction, and agarose gel elecrophoresis was used for detection of ITD mutation. ITD size was determined by sequencing analysis. For NPM1 mutation detection, we amplified genomic DNA by PCR using primers as previously reported. We found 11(22%) patients with FLT3/ITD mutation. ITDs were associated with higher WBC count(median of 90.7×109/L versus 5.85×109/L; p<.005) and higher peripheral blast percentage(median of 60% versus 39%; p=.016). OS was somewhat poorer for ITD positive patients compared with ITD negative patients(p=.12). FLT3/D835 mutations were found in 2(4%) of 51 patients. There was no case that had both FLT3/ITD and FLT3/D835 mutations. We next examined whether ITD size had an impact on clinical outcomes. ITD size was detectable in 9 out of 11 cases. Median ITD size was 33bp(range, 21–75bp). FLT3/ITD size was not significantly correlated with WBC count(p=.905), marrow(p=.730) or peripheral blast percentage(p=.413). Then we classified these FLT3/ITD mutations into long(≥40bp;4 cases) and short ITD(<40bp; 5 cases). Previous study pointed out that increasing ITD size might be associated with OS or RFS, but in this study we didn’t find that increasing ITD size was not associated with OS(p=.71). ITD size was not significantly correlated with NPM1 mutations. NPM1 mutations were found in 7(14%). Normal karyotype were found in 5(71%). Both FLT3/ITD and NPM1 mutation were found in 3 patients,a nd all died in early clinical courses. Previous reports pointed that NPM1 mutation was a favorable prognostic factor for overall survival(OS), but in this study, 5 year OS was 14% and worse compared to other studies. In FLT3/ITD positive case, statistical significance were not shown with or without NPM1 mutation(p=0.74). FLT3/ITD and NPM1 dual positive cases seems to be very poor prognosis, and intensive or aggressive therapy plans including allo bone marrow transplantation are needed for improving prognosis.

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Angioimmunoblastic T-cell lymphoma is a rare and unique disease with peculiar clinical features among malignant lymphomas. We report here a case of severe pulmonary arterial hypertension appearing as the main manifestation of angioimmunoblastic T-cell lymphoma in a 55-year-old woman. The patient was treated with the THP-COP regimen, consisting of pirarubicin, cyclophosphamide, vincristine, and prednisolone for 1 cycle, followed by the CHOP regimen, including doxorubicin instead of pirarubicin, for 5 cycles. Soon after the initiation of chemotherapy, all the patient's symptoms and abnormal findings, including an elevated pulmonary artery pressure, completely disappeared. As of 3 months after the end of therapy, the patient remains in complete remission. To our knowledge, this is the first report of pulmonary arterial hypertension secondary to angioimmunoblastic T-cell lymphoma.