Coexistent hairy cell leukaemia and hepatosplenic T-cell lymphoma: a case report.

BACKGROUND: Hairy cell leukaemia (HCL) is a chronic B-cell leukaemia characterized by expansion of neoplastic cells in the spleen, bone marrow and blood. Symptoms of HCL are related to pancytopenia and immune deficiency. Patients with HCL have an increased risk of second malignancy either in a form of synchronous disease or in a form of an increased incidence of a second neoplasm after the treatment of HCL. Hepatosplenic T-cell lymphoma (HSTCL) is a rare form of aggressive extranodal T-cell lymphoma. Its pathogenesis is connected to a chronic immune deficiency status and its coexistence with other neoplasms is practically non-existent. CASE: We present a case of a 53-year-old female patient suffering from hepatosplenomegaly, peripheral lymphadenopathy and related B symptoms. An excisional biopsy of the enlarged axillary lymph node revealed partial infiltration with CD3+/CD56+/TIA + T cell lymphoma. Bone marrow trephine biopsy and flow cytometric immunophenotypization of bone marrow cells and peripheral blood showed presence of two types of neoplastic cells in the peripheral blood and in the bone marrow (composite lymphoma). One of them showed typical morphologic characteristics and immunohistochemical features of HCL, while another one was morphologically and immunophenotypically consistent with the diagnosis of HSTCL, respectively. The patient was treated with multivalent chemotherapy including rituximab but all treatments turned out to be only partially effective. While HCL responded to the treatment, HSTCL was refractory to the chemotherapy and the patient died 7 months after the initial diagnosis because of haematemesis induced by Mallory-Weiss syndrome. CONCLUSION: This is the first recorded case of coexistent HCL and HSTCL in the same patient. A multidisciplinary approach, encompassing careful morphology interpretation, immunophenotypic, cytogenetic and molecular analyses, is mandatory to obtain an accurate diagnosis of composite lymphoma. VIRTUAL SLIDES: The virtual slides for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/9354870531161685.

Eleven years of experience with AIDS-related lymphomas at the Institute of Oncology Ljubljana.

Neoplastic diseases are currently recognized in more than 40% of HIV sero-positive patients, with AIDS-related lymphomas (ARL) being most common. A survey of the ARLs in patients attending our institute from 1998 to 2009 is given along with the clinical and pathological manifestations, treatment and outcomes. We conducted a retrospective chart review of patients treated for ARL between 1998 and 2009 at the Institute of Oncology Ljubljana. Patients were identified from the lymphoma database of our institute. Eight patients with ARL were diagnosed and treated at our Institute according to the then protocol for HIV sero-negative lymphoma patients. Seven patients received HAART. Six patients received prophylaxis for opportunistic infections and febrile neutropenia (FN), and 6 of them had at least one episode of FN. Seven patients are still alive and in complete remission 7-142 months after the diagnosis of ARL. One of the patients died of progressive lymphoma. The low incidence of ARL in the HIV-infected population, early stages at the presentation and the good response to treatment are not in accordance with the data from the literature. Whether this goes on account of the HAART treatment, prophylaxis for opportunistic infections and FN or a better follow-up of these patients remains to be answered. Also, the group is very small to make any definitive conclusions.

A single-center study of treatment outcomes and survival in patients with primary gastric lymphomas between 1990 and 2003.

Primary gastric lymphomas are the most common extranodal non-Hodgkin's lymphomas and are divided into indolent (low grade) and aggressive (high grade) types. They are mainly the disease of middle age, with a male predominance reported by most of the studies. For several years, surgery played a central role in diagnosis, staging, and treatment of this entity, yet recently there has been a move away from a surgical approach to conservative treatment. To determine the role of surgery as the initial treatment modality, we performed this retrospective single-center research on 245 patients with primary gastric lymphoma who were treated according to our protocol between 1990 and 2003. The patients' characteristics, distribution of histological types, treatment results, and disease-specific survival were followed. According to the histology, 59.2% had diffuse large B-cell lymphoma (DLCL), 26.1% MALT lymphoma, 9.8% mixed lymphoma (indolent and aggressive at the same time), while other types were infrequent. In total, 161 patients (65.7%) were treated with surgical resection as the initial treatment, which was then followed or not by additional therapy (chemotherapy, chemotherapy and radiotherapy, radiotherapy) depending on the histological type of lymphoma and the extent of residual disease after surgery. In 84 patients (34.3%), the treatment approach was conservative. The selection of treatment (chemotherapy, chemotherapy and radiotherapy, radiotherapy or Helicobacter pylori eradication only) was based on the histological type of lymphoma, considering also the patients' physical condition. The disease-specific survival in the group of patients who underwent surgery was statistically significantly better than in patients who were treated conservatively (p=0.049). At 5 years, it was 96.9% for the group treated with surgery and 89.8% in patients treated conservatively. However, the results were biased, as the patients who were treated conservatively were either in a worse performance status or presented with a more extensive disease. Similarly, in the DLCL type the disease-specific survival was better in the surgically treated group (97.2%) than in the conservatively treated patients (89.2%). The difference was barely significant (p=0.046) and again the results have to be considered with caution due to the selection of patients in a worse performance status or with a more extensive disease for conservative treatment. In the MALT lymphoma and mixed lymphoma types, there were no differences in the disease-specific survival between both treatment groups. Regarding the statement that for conservative treatment patients were selected who were unsuitable for the resection on account of concomitant diseases or due to the fact that the process was inoperable, we believe that the conservative approach gives comparable outcomes to the approach including initial surgery. The existing evidence thus no longer justifies surgery as the standard initial treatment and preference should be given to conservative treatment approaches.

Combined-modality therapy for clinical stage I or II Hodgkin's lymphoma: long-term results of the European Organisation for Research and Treatment of Cancer H7 randomized controlled trials.

PURPOSE: In early-stage Hodgkin's lymphoma (HL), subtotal nodal irradiation (STNI) and combined chemotherapy/radiotherapy produce high disease control rates but also considerable late toxicity. The aim of this study was to reduce this toxicity using a combination of low-intensity chemotherapy and involved-field radiotherapy (IF-RT) without jeopardizing disease control. PATIENTS AND METHODS: Patients with stage I or II HL were stratified into two groups, favorable and unfavorable, based on the following four prognostic factors: age, symptoms, number of involved areas, and mediastinal-thoracic ratio. The experimental therapy consisted of six cycles of epirubicin, bleomycin, vinblastine, and prednisone (EBVP) followed by IF-RT. It was randomly compared, in favorable patients, to STNI and, in unfavorable patients, to six cycles of mechlorethamine, vincristine, procarbazine, prednisone, doxorubicin, bleomycin, and vinblastine (MOPP/ABV hybrid) and IF-RT. RESULTS: Median follow-up time of the 722 patients included was 9 years. In 333 favorable patients, the 10-year event-free survival rates (EFS) were 88% in the EBVP arm and 78% in the STNI arm (P = .0113), with similar 10-year overall survival (OS) rates (92% v 92%, respectively; P = .79). In 389 unfavorable patients, the 10-year EFS rate was 88% in the MOPP/ABV arm compared with 68% in the EBVP arm (P < .001), leading to 10-year OS rates of 87% and 79%, respectively (P = .0175). CONCLUSION: A treatment strategy for early-stage HL based on prognostic factors leads to high OS rates in both favorable and unfavorable patients. In favorable patients, the combination of EBVP and IF-RT can replace STNI as standard treatment. In unfavorable patients, EBVP is significantly less efficient than MOPP/ABV.

Involved-field radiotherapy for advanced Hodgkin's lymphoma.

BACKGROUND: The use of involved-field radiotherapy after chemotherapy for advanced Hodgkin's lymphoma is controversial. METHODS: We randomly assigned patients with previously untreated stage III or IV Hodgkin's lymphoma who were in complete remission after hybrid chemotherapy with mechlorethamine, vincristine, procarbazine, prednisone, doxorubicin, bleomycin, and vinblastine (MOPP-ABV) to receive either no further treatment or involved-field radiotherapy. Radiotherapy consisted of 24 Gy to all initially involved nodal areas and 16 to 24 Gy to all initially involved extranodal sites. Patients in partial remission were treated with 30 Gy to nodal areas and 18 to 24 Gy to extranodal sites. RESULTS: Of 739 patients, 421 had a complete remission; 161 of these patients were assigned to no further treatment, and 172 to involved-field radiotherapy. The median follow-up was 79 months. The five-year event-free survival rate was 84 percent in the group that did not receive radiotherapy and 79 percent in the group that received involved-field radiotherapy (P=0.35). The five-year overall survival rates were 91 and 85 percent, respectively (P=0.07). Among the 250 patients in partial remission after chemotherapy, the five-year event-free and overall survival rates were 79 and 87 percent, respectively. CONCLUSIONS: Involved-field radiotherapy did not improve the outcome in patients with advanced-stage Hodgkin's lymphoma who had a complete remission after MOPP-ABV chemotherapy. Radiotherapy may benefit patients with a partial response after chemotherapy.