Clinical Analysis of 10 Cases of B Cell Lymphoma-Associated Hemophagocytic Syndrome / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To investigate the clinical and laboratory features of patient with B cell lymphoma associated hemophagocytic syndrome(B-LAHS).</p><p><b>METHODS</b>The clinical data of 10 cases of B-LAHS were retrospectively analysed and the relevant literatures were reviewed.</p><p><b>RESULTS</b>The median age of 10 cases diagnosed as B-LAHS was 55.5 (31-88) years old, and median time from attack to diagnosis was 2 months (2 weeks-4 months). The diagnosis can be made histopathologically and immunohistochemically by bone marrow biopsy. Among them 7 cases were diagnosed as large B cell lymphoma, 2 cases as mantle cell lymphoma and 1 case as small B cell lymphoma. The prominent clinical symptoms and signs were persistent fever (100%) and splenomegaly(90%), and the involvements with respiratory and digestive system were common. Another 1 case had systemic muscle pain and lactic acidosis as the first onset. Laboratory studies showed hepatic dysfunction, significantly elevated ferritin and lactate dehydrogenase, abnormal lymphocytes in peripheral blood smear, and hemophagocytosis in bone marrow smear. The FSC/SSC abnormalities of cloned B lymphoma cells were detected through flow cytometry (FCM). The complete remission (CR) was maintained in 4 cases receiving immunochemotherapy based on rituximab.</p><p><b>CONCLUSION</b>B-LAHS possesses heterogeneous clinical manifestations and rapid deterioration. Bone marrow biopsy and immunohistochemical examination can confirm the diagnosis. FCM may improve the early diagnosis of B-LAHS.</p>

Lymphocytic Clonal Expansion in Adult Patients with Epstein-Barr Virus-Associated Lymphoproliferative Disease / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To explore the lymphocytic clonal expansion in adult patients with Epstein-Barr virus-associated lymphoproliferative diseases (EBV+LPD), and to investigate the experimental methods for EBV+LPD cells so as to provide a more objective measure for the diagnosis, classification and prognosis in the early stage of this disease.</p><p><b>METHODS</b>Peripheral blood samples from 5 patients with EBV+LPD, 4 patients with adult infectious mononucleosis(IM) as negative control and 3 patients with acute NK-cell leukemia(ANKL) as positive control were collected. Prior to immunochemotherapy, viral loads and clonality were analysed by flow cytometry (FCM), T cell receptor gene rearrangement (TCR) was detected by real-time polymerase chain reaction (RT-PCR), and diversity of EB virus terminal repeat (EBV-TR) was detected by Southern blot.</p><p><b>RESULTS</b>FCM showed only 1 case with clonal TCRVβ in 5 patients with EBV+LPD, TCR clonal expansion could be detected both in patients with IM(4 of 4) and 4 patients with EBV+LPD(4 of 5), Out of patients with EBV+LPD, 1 patient displayed a monoclonal band and 2 patients showed oligoclonal bands when detecting EBV-TR by southen blot.</p><p><b>CONCLUSION</b>Detecting the diversity of EBV-TR by Southern blot may be the most objective way to reflex clonal transformation of EBV+LPD, which is of great benefit to the diagnosis, classification and prognosis in the early stage of this disease.</p>

Clinical features and laboratory findings of adult Epstein-Barr virus associated T/NK lymphoproliferative disease / 中国实验血液学杂志

The aim of this study was to analyze the clinical features and laboratory findings of adult Epstein-Barr virus associated T/NK cell lymphoproliferative disease (EBV+T/NK-LPD) and to investigate the early diagnosis and prognosis of EBV+T/NK-LPD. The clinical data of 19 adult patients with EBV+T/NK-LPD were retrospectively analyzed. The results indicated that there were 11 males and 8 females. The median age was 32 years (range: 20-70 years). The average duration from onset of symptoms to diagnosis was 3.5 months. The median survival time was 2.5 months. Unkown fever, hepatosplenomegaly, liver dysfunction and interstitial pneumonia were the main clinical features. High levels of β2-MG, LDH, TNF, IL-6 and significantly increased EBV-DNA level (median level > 10(6) copies/ml) were occurred in all the patients. Cytopenia was seen in 18 cases. Morphologically, atypical large granular lymphocytes and hemophagocytosis were common in bone marrow smears. Deletion of CD5 or CD7 were frequently observed in T/NK lymphocytes in bone marrow cells by flow cytometry. Bone marrow biopsy showed atypical lymphocyte interstitial infiltrated in 10 cases, while a few large cells infiltrated in 6 cases. Immunohistochemistry showed the expression of CD3(+)CD56(+) were seen in 2 cases, CD3(+)CD8(+) in 11 cases and CD3(+)CD4(+) in 3 cases. TIA-1 and EBER were positive in all biopsy specimens. Three cases underwent biopsy of lymph nodes showed reactive proliferations of lymphocytes. All the patients died of multiorgan failure. It is concluded that the fever, hepatosplenomegaly are the most common clinical features in adult EBV+T/NK-LPD, the bone marrow infiltration of EBV-infected T/NK lymphocytes and significantly increased EBV-DNA level can be observed in all cases, the clinical outcome of this disease is poor, these clinical and experimental features can be served as a reliable marker for the timely diagnosis of adult EBV+T/NK-LPD.

Clinical analysis of 10 patients with de novo CD5 positive diffuse large B cell lymphoma / 中国实验血液学杂志

To explore the clinical characteristics, diagnosis, treatment outcome and prognosis of de novo CD5 positive diffuse large B cell lymphoma (CD5(+)DLBCL), clinical data of 10 patients with pathologically confirmed CD5(+)DLBCL were retrospectively analyzed. The results indicated that 9 out of 10 patients were older than 60 years. All cases were in III/IV stages according to Ann-Arbor Staging System. Bone marrow biopsy with immunohistochemistry showed lymphoma involvement in 5 cases. Nine patients received chemotherapy with anti-CD20 monoclonal antibody (Rituximab) except one. Five cases achieved CR, two cases achieved PR, two cases achieved SD, one case achieved PD. Eight cases died within 2 years because of relapse or disease progression, in which 3 cases developed central nervous system lymphoma. The median survival time was 16 (1-23) months, 2-year survival rate was 20.40%. It is concluded that de novo CD5(+) DLBCL is rare in clinic, but it is a kind of highly aggressive lymphoma with poor prognosis. So, new treatment strategy should be explored.

Archive analysis of 1056 patients with acute leukemia / 白血病·淋巴瘤

Objective To explore the features of patients with acute leukemia (AL) in Hematology Department of Shenzhen Hospital affliated to Beijing University during the past six years. Methods The data of the 1056 acute leukemia dignosed in our department between 2002-2007 were collected and analyzed retrospectively. Results The male patients was predominant (male/female ratio was 1.78). The number of patients with acute myeloid leukemia(AML) was higher than that of patients with acute lymphoblastic leukemia (ALL) (AML/ALL ratio was 1.85). Most of the AML patients were young adult (65 %) and elderly ALL patients were very rare(1.33 %). AL cases were tended to increase gradually by year. The ratio of treatment-related AL in Shenzhen was lower than that reported literature in other countries. The major cause of the treatment-related AL were chemotherapy/radiotherapy and psoriasis treated with bimolane. Conclusion Some of the features of AL patients in China were further confirmed and some new features were also found in this study. These changes should be demonstrated by the next national epidemiology survey of leukemia.