Rosa Ruchlemer

Long remissions in hairy cell leukemia with purine analogs - A report of 219 patients with a median follow-up of 12.5 years

Both pentostatin and cladribine have efficacy in hairy cell leukemia (HCL), but it is not known which agent achieves better results.

Diagnostic accuracy of PET⁄CT in patients with extranodal marginal zone MALT lymphoma

Background:  18Fluoro‐2‐deoxyglucose (18FDG) positron emission tomography (PET) is widely used for initial staging and follow‐up in patients with malignant lymphoma. While earlier studies suggested a limited role for PET in extranodal marginal zone mucosa‐associated lymphoid tissue (MALT) lymphoma patients due to their non‐FDG avidity, more recent reports have suggested that the issue is controversial. In the present study, we evaluated the diagnostic accuracy of PET integrated with CT (PETCT) in patients with MALT lymphoma and assessed its reliability in clinical staging and monitoring response.

B-prolymphocytic leukaemia with t(11;14) revisited: a splenomegalic form of mantle cell lymphoma evolving with leukaemia

We reviewed eight cases that were diagnosed before 1995 with B‐prolymphocytic leukaemia (B‐PLL) harbouring t(11;14)(q13;q32) and/or cyclin D1 staining. Thirteen B‐PLL patients without t(11;14) were selected as controls. Peripheral blood, bone marrow and histological sections were re‐examined without cytogenetic information. Final diagnosis was made using morphology, cytogenetics, immunophenotype and immunohistochemistry. Clinical characteristics were similar for both groups except for younger age, male predominance and extranodal involvement in cases with t(11;14). CD5 was more frequently positive in the t(11;14)+ group (80%) than in the t(11;14)− group (31%). Surface membrane immunoglobulin was strongly expressed by all t(11;14)+ cases, but only 45% of t(11;14)− cases. Histopathological and cytological review of cases with t(11;14) showed an infiltrate with a mixture of cells, some resembling prolymphocytes and others with mantle cell lymphoma (MCL) morphology. Blood films of cases with t(11;14) showed features suggestive of B‐PLL in three, and in others, a mixture of cells resembling MCL and nucleolated ones; none corresponded to the blastoid form of MCL. We suggest that ‘B‐PLL’ with t(11;14) may represent a splenomegalic form of MCL evolving with leukaemia. These cases illustrate the importance of tissue diagnosis with cyclin D1 staining and fluorescence in situ hybridization analysis in B‐cell leukaemia with prolymphocytic features.

Alpha-interferon-induced arthritis: clinical presentation, treatment, and prevention

OBJECTIVE The therapeutic applications of alpha-interferon (IFN) have expanded greatly to include chronic viral hepatitis and malignant disorders. Autoimmune phenomena occur frequently with IFN therapy, but arthritis is uncommon. We describe the clinical features and treatment of IFN-induced arthritis. METHODS A patient with chronic myelogenous leukemia who developed arthritis secondary to IFN therapy is presented. The clinical features and treatment of this condition in 37 additional cases are reviewed. RESULTS The most common clinical presentation was symmetric polyarthritis. This was associated with antinuclear antibodies in 72% of patients and rheumatoid factor in 34%. Cessation of IFN, with or without the addition of antiinflammatory or remittive agents, resulted in remission of arthritis in 89% and 71% of the cases, respectively. Restarting IFN therapy resulted in recurrence of arthritis in 63%. In the patient described in this report, recurrence of arthritis was prevented by coadministration of hydroxychloroquine (HCQ) and prednisone. CONCLUSION Arthritis is an uncommon complication of IFN therapy; but it may lead to cessation of this treatment modality. In such cases, coadministration of a remittive agent such as HCQ may enable reinstitution of IFN therapy without recurrence of arthritis.

Splenectomy in mantle cell lymphoma with leukaemia: a comparison with chronic lymphocytic leukaemia

Summary. We reviewed data on 63 patients with mantle cell lymphoma (MCL) with leukaemia (n = 16) and chronic lymphocytic leukaemia (CLL, n = 47), splenectomized over a 10‐year period. Primary indications for surgery were cytopenia(s) or autoimmune phenomena and progressive or refractory disease with splenomegaly. The spleens removed were on average larger in MCL (median 2·6 kg) than in CLL (1·0 kg). Splenectomy improved the blood counts in 62% of patients with MCL and 47% with stage C CLL, both with cytopenias. The MCL patients showed a decrease in the leucocytosis (medians 60·3–29·1 × 109/l before and after splenectomy), whereas there was an increase in the leucocytosis in CLL (medians 24·2–44 × 109/l). With a median follow up post splenectomy of 10 months (range: < 1–128), 18 patients (four MCL and 14 CLL) have not required further therapy for up to 66 months. We conclude that splenectomy is a useful treatment in MCL and advanced CLL for the correction of cytopenias, reducing the leucocyte count and allowing prolonged periods of clinical remission without therapy. Differences seen between MCL and CLL in spleen size, and in response of the leucocytosis suggest a central role for the spleen in the evolution of MCL with leukaemia.

Richter's transformation to diffuse large B-cell lymphoma: A retrospective study reporting clinical data, outcome, and the benefit of adding rituximab to chemotherapy, from the Israeli CLL Study Group

Richters syndrome (RS) is the rare development of an aggressive lymphoid malignancy in a patient with pre‐existing chronic lymphocytic leukemia (CLL). Data on RS is sparse and mostly derived from case reports or small series of patients and only a few larger cohorts have been published. The purpose of this large retrospective study was to summarize our national experience with RS in CLL, examine possible risk factors, and analyze relevant demographic, laboratory and clinical parameters, including results of therapy and outcome. We first evaluated data obtained from 119 patients with RS diagnosed during 1971–2010 from 12 medical centers in Israel. The final cohort summarized consisted of 81 patients with RS who developed only diffuse large B‐cell lymphoma (DLBCL) after exclusion all cases with insufficient data and those who were not DLBCL. Median overall survival from time of diagnosis of RS was 8 months; after applying the Richter score, patients could be stratified into three prognostic groups, while all other clinical and laboratory parameters evaluated had no prognostic significance. Prior therapy for CLL had no impact on RS survival (P = 0.8) and patients with therapy “naïve” RS and those who had already received chemotherapy prior to developing RS, had the same survival. The addition of rituximab to chemotherapy for RS improved 2 years overall survival from 19% in the chemotherapy alone arm to 42% (P value of 0.001). Although prognosis of patients with RS remains dismal, this retrospective observation provides support for the use of chemo‐immunotherapy in DLBCL‐RS. Am. J. Hematol. 89:E218–E222, 2014.

Geography, ethnicity and “roots” in chronic lymphocytic leukemia

Abstract Chronic lymphocytic leukemia (CLL) is the most common leukemia in Western countries including Israel, and is less frequent in the Orient, Asia and Middle Eastern Arabic speaking countries. These trends persist in migrants to other countries, continuing into subsequent generations. Biological and genetic disparities have been reported for different ethnic groups. The absence of an association between place of birth and the occurrence of CLL is more in line with a genetic basis for the geographic variations in incidence. Genetic predisposition to CLL is supported by the documented familial aggregation of CLL, with an increased frequency of 8.5-fold among first-degree relatives of patients with CLL and the detection of CLL-like clones in 13.5% of first-degree relatives. It is likely that the development of CLL depends on the interplay of a genetic predisposition with exposure to environmental factors. To better understand the interplay of ethnicity and CLL we reviewed all the available literature on ethnic specific differences for this common form of leukemia.

Inhaled medicinal cannabis and the immunocompromised patient

Medicinal cannabis is an invaluable adjunct therapy for pain relief, nausea, anorexia, and mood modification in cancer patients and is available as cookies or cakes, as sublingual drops, as a vaporized mist, or for smoking. However, as with every herb, various microorganisms are carried on its leaves and flowers which when inhaled could expose the user, in particular immunocompromised patients, to the risk of opportunistic lung infections, primarily from inhaled molds. The objective of this study was to identify the safest way of using medicinal cannabis in immunosuppressed patients by finding the optimal method of sterilization with minimal loss of activity of cannabis. We describe the results of culturing the cannabis herb, three methods of sterilization, and the measured loss of a main cannabinoid compound activity. Systematic sterilization of medicinal cannabis can eliminate the risk of fatal opportunistic infections associated with cannabis among patients at risk.

Insect-bite-like reaction in patients with chronic lymphocytic leukemia: a study from the Israeli Chronic Lymphocytic Leukemia Study Group.

An insect‐bite‐like reaction is known to occur in patients with chronic lymphocytic leukemia (CLL). Most of the literature, however, consists of isolated case reports or small case series. The aim of this retrospective study was to review the national experience with insect‐bite‐like reaction in a large group of patients with CLL. The study cohort of patients with these skin reactions consisted of 48 patients (25 males, 23 females) of mean age 64.8 yr (range 33–89) at skin eruption. Data on clinical, histologic, immunophenotypic, and cytogenetic characteristics, treatment, and outcome were collected from the medical files. Mean time between diagnosis of CLL and appearance of the skin lesions was 3.1 yr (range −4 to 14 yr). The eruption was not related to disease activity or the course of the hematological disease. The eruption preceded the diagnosis of CLL in 10 patients (by 0–4 yr); and followed the diagnosis in 36; in 11 patients, it occurred during therapy for CLL and in nine after therapy. Mean duration of the skin findings was 21.5 months (range 0.3–132). The eruption usually presented in summer, although it occurred also at other times of the year, and predominantly affected the upper and lower limbs, although it also appeared on unexposed areas. Treatment included local ointments, antihistaminics, oral steroids, antibiotics, phototherapy, and dapsone with varying responses. Insect‐bite‐like reactions is a relatively common and disturbing skin reaction in CLL patients, it may be related to the immune dysregulation accompanying CLL and further exacerbated by external factors, including actual insect bites, chemoimmunotherapy, and pyogenic infection.

Iron deficiency anemia at admission for labor and delivery is associated with an increased risk for Cesarean section and adverse maternal and neonatal outcomes.

Maternal iron deficiency anemia (IDA) impacts placenta and fetus. We evaluated effects of IDA at admission for delivery on cesarean rates, and adverse maternal and neonatal outcomes.