Anaplastic large cell lymphoma (ALCL) is a distinct type of T/null-cell non-Hodgkin lymphoma that commonly involves nodal and extranodal sites. The World Health Organization of lymphoid neoplasms recognizes two types: anaplastic lymphoma kinase (ALK) positive or ALK negative, the former as a result of abnormalities involving the ALK gene at chromosome 2p23. Patients with ALCL rarely develop a leukemic phase of disease, either at the time of initial presentation or during the clinical course. Described herein is a patient with ALK+ ALCL, small cell variant, associated with the t(2;5)(p23;q35), who initially presented with leukemic involvement and an extraordinarily high leukocyte count of 529 x 10(9)/L, which subsequently peaked at 587 x 10(9)/L. Despite chemotherapy the patient died 2(1/2) months after diagnosis. In the literature review 20 well-documented cases are identified of ALCL in leukemic phase reported previously, with a WBC ranging from 15 to 151 x 10(9)/L. Leukemic phase of ALCL occurs almost exclusively in patients with ALK+ ALCL, most often associated with the small cell variant and the t(2;5)(p23;q35), similar to the present case. Patients with leukemic phase ALK+ ALCL appear to have a poorer prognosis than most patients with ALK+ ALCL.

T-cell prolymphocytic leukemia (T-PLL) can involve extramedullary sites, but the diagnosis is usually established by examination of blood and bone marrow. As a result, the histologic findings at extramedullary sites are poorly documented in the literature. We describe 19 extramedullary biopsy specimens from 14 patients with T-PLL. Skin (n = 10) was the most common site biopsied. T-PLL surrounded dermal blood vessels and appendages (n = 6), diffusely replaced dermis (n = 3), or formed a subcutaneous mass (n = 1). Other extramedullary sites included liver and lymph nodes (3 each) and spleen, lung, and cecum (1 each). In liver and lymph nodes, the neoplasm predominantly involved portal tracts and paracortex, respectively. Cytologically, the T-PLL cells were round (n = 16) or Sezary cell-like (n = 3). Nucleoli were observed in a subset of cells in 8 specimens and were prominent in 3 specimens. Immunostaining for T-cell leukemia-1 (TCL-1) was positive in specimens from 9 (64%) of 14 patients. We conclude that the prolymphocytoid features of T-PLL cells can be difficult to detect in routinely stained sections of extramedullary biopsy specimens. TCL-1 expression can aid in diagnosis at extramedullary sites.

Most human cases of West Nile virus infection are acquired via bites from an infected mosquito. In some cases, infection may also be transmitted by infected blood products or transplanted organs. There have been recent publications suggesting that chemotherapy and immunosuppression may increase a person's risks of developing central nervous system disease if the person is infected with the West Nile virus. Because patients undergoing hematopoietic stem cell transplantation not only are immunocompromised, but also receive multiple blood products, they are at a particularly high risk for acquiring symptomatic disease if exposed to the West Nile Virus. We describe here 2 patients who underwent hematopoietic transplantation at our institution and subsequently developed fatal West Nile virus infections.

Two cases of Langerhans cell histiocytosis involving the vulva are reported. The clinical features of Case 1 have been previously reported. The patients, aged 31 (Case 1) and 52 (Case 2) years, had disease limited to the vulva at the time of diagnosis. In both cases, the vulvar lesions were composed of Langerhans cells with twisted nuclei and nuclear grooves, associated with eosinophils and other inflammatory cells. Immunohistochemical studies using fixed, paraffin-embedded tissue sections showed that the Langerhans cells were positive for CD1A and S-100 protein in both cases, supporting the morphologic diagnosis. The first patient had multiple local recurrences during 3 years despite radiation therapy and vulvectomy, but is currently in remission and being treated with thalidomide. The second patient experienced disseminated disease involving multiple bony sites and the mouth despite radiation therapy. Including the present two cases, 6 of 18 (33%) cases of isolated vulvar LCH subsequently disseminated, most commonly to bone; other patients had local recurrences. Thus isolated vulvar LCH has the potential for aggressive clinical behavior, either as local recurrence or disseminated disease. New treatment modalities are needed for this disease.

The objective of this study is to assess the efficacy of ICRF-187 as a protective agent against anthracycline cardiotoxicity. Cardiac function was evaluated by echocardiography before and after each cycle of anthracycline chemotherapy associated with ICRF-187 and compared with that of a second group receiving anthracycline chemotherapy without ICRF-187. The patients were a group of 15 consecutive children affected with various types of solid tumors who were treated with either doxorubicin-daunomycin or epirubicin (average doses 340 and 280 mg/m2, respectively), and treatment was associated with ICRF-187. A second group of 15 consecutive children affected with different malignancies were simultaneously treated with either doxorubicin-daunomycin or epirubicin (average doses 309 and 270 mg/m2, respectively), but without ICRF-187 association. None of the patients treated with anthracyclines and ICRF-187 association showed abnormalities on echocardiographic examination. In the second group of patients treated with anthracyclines but without ICRF-187 association, we observed a decrease in the left ventricular ejection fraction to < 55% and a decrease in the left ventricular fractional shortening to < 28% in two patients (13.3%). One of these (6.6%) showed a dilatative cardiomyopathy. Both groups of patients were treated with low doses of anthracyclines. Although this study was not randomized, in patients without ICRF-87 cardioprotection, there was a trend for a worse evolution with one case of clinical cardiomyopathy as well as subclinical cardiac abnormalities.

Surgery is the treatment of choice for low-grade astrocytoma while radiotherapy is carried out only when total resection is not possible. This study assessed the effectiveness of chemotherapy in nonresectable cases. Thirteen children with nonresectable astrocytoma were treated with carboplatin and etoposide and after four cycles the response to treatment was evaluated according to radiologic criteria. The results were: one with complete response (CR), three with minor response (MR), six with stable disease (SD), and three with progressive disease (PD). Moreover, in 77% there was an improvement in the neurologic picture. In particular, two cases with hypothalamic astrocytoma showed a regression of the diencephalic syndrome following chemotherapy. In six cases chemotherapy was carried out, at reduced dosage, after the first four cycles either because there was clinical improvement or because it was necessary to postpone radiotherapy in very young patients. After a follow-up period ranging between 11 and 63 months (average: 30 months), nine of the 13 patients are alive (69%) while four died of disease progression. Further studies would be useful to evaluate the role of chemotherapy in the management of low-grade astrocytoma.

Context.—Pleural involvement by lymphoma is relatively common. However, there are very few clinicopathologic studies reported in the literature of lymphomas involving the pleura. Objective.—To characterize the clinicopathologic features of lymphomas involving the pleura. Design.—We reviewed the clinicopathologic features of 34 patients with lymphoma involving the pleura proven by biopsy and classified these neoplasms using the World Health Organization classification. Results.—There were 22 men and 12 women, with an average age of 62 years (range, 22–82 years). Nine (26.5%) patients had pleural involvement as the only site of disease, 22 (64.7%) had other sites of involvement, and 3 (8.8%) had inadequate staging data. Eighteen (56.2%) of 32 patients with adequate clinical data had a history of lymphoma (including 3 patients with pleural involvement as the only disease site). In 29 (85.3%) cases, a specific diagnosis according to the World Health Organization classification could be made: 17 (58.6%) diffuse large B-cell lymphoma, 5 (17.2%) follicular lymphoma (including a case with areas of diffuse large B-cell lymphoma), 2 (6.9%) small lymphocytic lymphomas/chronic lymphocytic leukemia, 2 (6.9%) precursor T-cell lymphoblastic lymphoma/leukemia, 1 (3.4%) mantle cell lymphoma, 1 (3.4%) posttransplant lymphoproliferative disorder, and 1 (3.4%) classical Hodgkin lymphoma. The other 5 cases were B-cell lymphomas that could not be further classified. Cytologic examination of pleural fluid was performed in 15 cases and was positive for lymphoma in 8 (53.3%) cases. Conclusions.—Most patients with lymphoma involving the pleura have simultaneous evidence of systemic involvement. The most frequent type is diffuse large B-cell lymphoma, followed by follicular lymphoma. Cytologic examination can have negative results in patients with pleural involvement by lymphoma.

Three children, treated with dactinomycin and vincristine without radiotherapy showed hepatic toxicity consistent with diagnostic criteria for hepatic veno-occlusive disease (VOD). Two patients were affected with Wilms' tumor and the third with malignant fibromatosis. The clinical manifestations of VOD were mild and regressed in all patients after supportive therapy. Serial ultrasonography (US) was performed in all cases and was useful in confirming the diagnosis and in evaluating the severity of the disease. US features of VOD were hepatomegaly, gallbladder wall thickening, ill-defined borders of the hepatic vessels, and ascites. Parenchymal heterogeneity was still present after several months as a result of the hepatic injury. The hazy appearance of the portal vessels and the gallbladder wall thickening seemed directly correlated with the degree of hepatic involvement in the early phase of VOD.