Hepatosplenic B-cell lymphoma associated with hemophagocytic syndrome: a case report

While T-cell non-Hodgkin's lymphoma (NHL) associated with hemophagocytic syndrome (HPS) has been frequently observed, B-cell NHL associated with HPS has been rarely reported. We report a case of hepatosplenic B-cell lymphoma associated with HPS in a 41-year-old woman who presented with fever of unknown origin. An abdominal CT scan revealed splenomegaly with focal splenic infarction. Splenectomy and a liver wedge biopsy showed sinusoidal-pattern infiltration of medium to large tumor cells with positive reaction to a B-lymphocyte marker. Findings on bone marrow examination showed proliferation of histiocytes with avid hemophagocytosis.

Clinical Effect of Granulocyte Transfusion Therapy in Neutropenia-Related Infection / 대한혈액학회지

BACKGROUND: After a zealous advocates of granulocyte transfusion therapy (GTX) in the 1970s and early 1980s, the use of GTX has diminished strikingly because of the several problems of GTX and the introduction of new antimicrobial agents and recombinant hematopoietic growth factors. Recently, GTX offers renewed interest because several investigators reported the transfusion efficacy of granulocytes collected by stimulating normal donors with recombinant human granulocyte-colony stimulating factor (G-CSF). METHODS: To evaluate the safety and efficacy of GTX, thirteen patients with neutropenia- related infections at Chonnam University Hospital from March 1997 to February 1998 were treated with dexamethasone- or G-CSF-stimulated granulocyte transfusions apheresed from normal donor. RESULTS: Patients received a mean number of 2.4 transfusions (range, 1-7) and a mean dose of 5.5x1010 granulocytes (range, 0.2-19.6). Six patients (46.2%) had favorable responses. Favorable responses occurred among patients with more fungal infection than bacterial infection (71.4 vs 28.6%, P<0.05) and more increment of absolute neutrophil count at 1 hour after transfusion (P<0.05). Adverse reactions of GTX were pulmonary edema in 2 patient (15.4%) and transient hypoxia in 1 patient (7.7%). One patient (7.7%) with pulmonary edema died of severe pulmonary reaction. Two of 20 donors received by G-CSF complained of mild myalgia and bone pain. CONCLUSION: G-CSF- or dexamethasone-stimulated GTXs were well tolerated and may be clinically beneficial for neutropenia-related infection, particularly in fungal infection, that is refractory to antimicrobial therapy.

Frequency of bcl-2 mbr/JH Rearrangement and Prognostic Implication of bcl-2, c-myc Protein Expression in Non-Hodgkin's Lymphoma / 대한혈액학회지

BACKGROUND: Deregulated bcl-2 appears to prolong cell survival and to cooperative with c-myc in promoting cell proliferation. We investigated whether there is any clinicopathologic correlation between the survival and the frequency of bcl-2/JH rearrangement and bcl-2, c-myc protein expression in non-Hodgkin's lymphoma. METHODS: We conducted a study for bcl-2 mbr/JH rearrangement with polymerase chain reaction and for bcl-2, c-myc expression with immunohistochemical staining in 46 formalin-fixed, paraffin-embedded non-Hodgkin's lymphoma tissues of patients treated with CHOP chemotherapy including 37 specimens of diffuse large cell type. RESULTS: The bcl-2 mbr/JH rearrangement was positive in 13% (6/46) of non-Hodgkin's lymphoma specimens. For bcl-2, strong positive reaction (3+) and 2+ positive reaction were seen in 16 (35%) and 16 cases (35%), respectively; while 3+ and 2+ reactions were found in 20 (44%) and 16 cases (35%), respectively, for c-myc by immunohistochemistry. Eighty one percent of positive cases for bcl-2 were also positive for c-myc simultaneously. The 6 cases with bcl-2 mbr/JH rearrangement were weakly positive at 3 cases and strong positive at 3 cases for bcl-2 by staining. In cases of diffuse large cell lymphoma, high expression (3+) of bcl-2 & c-myc protein tended to have a shorter 2 year survival, though it was statistically not significant. CONCLUSION: High expression of bcl-2 and c-myc protein suggest that bcl-2 cooperate with c-myc in tumorigenesis of aggressive non-Hodgkin's lymphoma. The prognostic implication of bcl-2 and c-myc expression in diffuse large cell lymphoma patients needs to be evaluated in a larger, prospective cohort to draw a definitive conclusion.

Treatment of Acute Promyelocytic Leukemia (APL) by a Combination of All-Trans Retinoic Acid (ATRA) and Chemotherapy / 대한혈액학회지

BACKGROUND: All-trans-retinoic acid (ATRA) induces complete remission (CR) in the great majority of patients with PML/RAR -positive acute promyelocytic leukemia (APL). However, it is associated with a rapid rise in leukocytes in one third to half the patients, with potentially fatal "ATRA syndrome". Furthermore, most of the patients relapse with maintenance therapy using ATRA alone or low-dose chemotherapy. In this study, we have analyzed the outcome for APL patients who were treated with ATRA alone or combined with low-dose chemotherapy followed by postremission chemotherapy in Chonnam University Hospital from April 1993 to December 1997. METHODS: Sixteen patients with newly diagnosed APL were eligible to analysis. Patients received 45mg/m2 ATRA until CR occurred. If initial WBC were above 5,000/microliter, low-dose chemotherapy was concomitantly given, and if during the ATRA therapy WBC were above 5,000/microliter by day 5 or 10,000/microliter by day 10, or 15,000/microliter by day 15, low-dose chemotherapy was added. Four polychemotherapy cycles or allogeneic bone marrow transplantation were given as postremission therapy. RESULTS: Median age was 34 years (range, 17 to 67). Of 16 APL patients, 15 (93.8%) achieved CR and 1 (6.2%) died of intracerebral hemorrhage. After a median follow-up of 11.5 months (range, 0 to 47), the Kaplan-Meier estimated overall survival (OS) rate was 87.1 +/- 8.6% at 3 year, the event-free survival (EFS) rate was 87.1 +/- 8.6%, 58.0 +/- 24.4% and 29.0 +/- 23.9% at 1 year, 2 year and 3 year, and the disease-free survival (DFS) rate was 92.9 +/- 6.9%, 69.6 +/- 20.7% and 46.4 +/- 23.5% at 1 year, 2 year and 3 year, respectively. CONCLUSION: The present study suggests that ATRA with or without low-dose chemotherapy followed by postremission chemotherapy is a well-tolerated and effective regimen that is shown to improve the CR rate, reduce a early mortality rate and considerably prolong the overall survival in patients with newly diagnosed APL.