Ewa Wasik-Szczepanek

ZAP-70 and CD38 expression are independent prognostic factors in patients with B-cell chronic lymphocytic leukaemia and combined analysis improves their predictive value.

Recently identified biological risk factors in B-cell chronic lymphocytic leukemia (B-CLL) include ZAP-70 and CD38 expression. The present study was conducted to clarify whether a combined analysis could improve predictive impact of these two parameters. We examined the expression of ZAP-70 and CD38 by flow cytometry method in 217 newly diagnosed, consecutive, unselected and well characterized B-CLL patients in relation to laboratory parameters and clinical outcome. We confirmed that both ZAP-70 as well as CD38 were independent of prognostic factors. There was a significant correlation between the percentage of leukemic cells positive for ZAP-70 and the percentage of CD38+CD19+ cells (R=0.629; p=0.000001). Combined analysis of ZAP-70 and CD38 showed concordant results in 158/217 patients (72.8%), while in 59 patients the results were discordant (27.2%). A mean treatment free survival (TFS) was the longest in ZAP-70-CD38-patients (45.6 months, comparing to 13.6 months in ZAP-70+CD38+ group). Also a mean overall survival was the longest in ZAP-70-CD38- patients (224.7 months compared to 77.9 months in ZAP-70+CD38+ patients).

Intravascular B-cell lymphoma in a 38-year-old woman: a case report

Abstract.Intravascular lymphoma (IVL) is a rare aggressive disease characterised by the presence of lymphoma cells only in the lumina of small vessels, particularly capillaries. Only about 200 cases have been reported in the world (some of them retrospectively). IVL is predominantly of B-cell lineage origin but occasionally T-cell lineage occurs. Multiple organs may be involved and a variety of clinical presentations have been described. These include nephrotic syndrome, pyrexia and hypertension, breathlessness and haemolytic anaemia, leukopoenia, pancytopoenia and disseminated intravascular coagulation. We report a case of a 38-year-old woman with a highly aggressive clinical course of IVL. She was admitted to the Department of Neurosurgery because of spondylolisthesis of L5-S1 qualified to surgery. During hospitalisation haemolytic anaemia, thrombocytopoenia and splenomegaly were observed and she was admitted to the Department of Haematology for diagnosis. During her staying in the hospital, new symptoms, such as kidney and liver failure, occurred and the central nervous system was involved. The clinical course was very rapid and progressive. Corticosteroid therapy was started but the disease soon led to the fatal outcome. Diagnosis was established at post-mortem examination.

Treatment of multiple myeloma patients with autologous stem cell transplantation — a fresh analysis

Patients with multiple myeloma (MM) treated with conventional chemotherapy have an average survival of approximately three years. High dose chemotherapy followed by autologous stem cell transplantation (ASCT), first introduced in the mid-1980s, is now considered the standard therapy for almost all patients with multiple myeloma, because it prolongs overall survival and disease free survival. Between November 1997 and October 2006, 122 patients with MM (58 females, 64 males, median age 51.0 years [± 7.98] range: 30-66 years) were transplanted in the Department of Hematooncology and Bone Marrow Transplantation at the Medical University of Lublin: 47 patients were in complete remission or in unconfirmed complete remission, 66 patients were in partial remission, and nine had stable disease. Of these, there were 95 patients with IgG myeloma, 16 with IgA myeloma, one with IgG/IgA, one with IgM myeloma, five with non secretory type, two with solitary tumor and two with LCD myeloma. According to Durie-Salmon, 62 patients had stage III of the disease, 46 had stage II and four had stage I. Most patients (69/122) were transplanted after two or more cycles of chemotherapy, 48 patients were transplanted after one cycle of chemotherapy, one patient after surgery and rtg- -therapy and four patients had not been treated. In mobilisation procedure, the patients received a single infusion of cyclophosphamide (4-6 g/m(2)) or etoposide 1.6 g/m(2) followed by daily administration of G-CSF until the peripheral stem cells harvest. The number of median harvest sessions was 2.0 (± 0.89) (range: 1-5). An average of 7.09 (± 33.28) × 106 CD34(+) cells/kg were collected from each patient (range: 1.8-111.0 × 10⁶/kg). Conditioning regimen consisted of high dose melphalan 60-210 mg/m(2) without TBI. An average of 3.04 (± 11.59) × 10⁶ CD34+ cells/kg were transplanted to each patient. Fatal complications occured in four patients (treatment- -related mortality = 3.2%). In all patients there was regeneration of hematopoiesis. The median number of days for recovery to ANC > 0.5 × 10⁹/l was 13 (± 4.69) (range: 10-38) and platelets recovery to > 50 × 10⁹/l was 25 days (± 11.65) (range: 12-45). Median time of hospitalization was 22 days (± 7.14) (range: 14-50). Patients were evaluated on day 100 after transplantation: 74.9% achieved CR and nCR, 14.3% were in PR, 5.4% had SD and 5.4% had progressed. Median of OS was 45 months (± 30.67). OS at 3-years was 84% and at 7-years 59%. Median PFS was 25 months (± 26.13). PFS at 3-years was 68%, and at 7-years was 43%. At present (November 2009) 52 patients (42%) are still alive. High-dose chemotherapy followed by autologous stem cell transplantation is a valuable, well tolerated method of treatment for patients with MM that allows the achievement of long- -lasting survival.

Therapy - Related Myeloid Neoplasm in a Patient with Prior Treatment for Chronic Lymphocytic Leukemia: A Case Report

Background: Acute myeloid leukemia or myelodysplastic syndrome, during the course of chronic lymphocytic leukemia, is a rare entity. In such a situation, most previous cases were therapy-related after treatment. However, in very rare cases, acute myeloid leukemia has been diagnosed in untreated patients with chronic lymphocytic leukemia. Materials and Methods: In this paper, we present the case-study of a 60-year old woman who was diagnosed six years previously as being afflicted with chronic lymphocytic leukemia with no chromosomal changes. She was successfully treated with fludarabine in combination with cyclophosphamide and rituximab, and she obtained complete remission, yet with incomplete recovery. Subsequently, three years later, she was re-admitted to hospital with deep pancytopenia. Her bone marrow biopsy then revealed that 52% of her blasts cells demonstrated a myelomonocytic immunophenotype, albeit with no chronic lymphocytic leukemia cell clone, showing the following complex chromosomal abnormalities: 45~47, XX, del(5)(q13q33), trisomy 8, trisomy 20, monosomy 21, +mar[cp12]/46, XX. Thus, a diagnosis was made of acute myelomonocytic leukemia preceded by a transforming myelodysplastic syndrome. The patient received induction chemotherapy and obtained complete remission. Unfortunately, one month after completion of her consolidation regimen, a relapse occurred. She died 8 months post-diagnosis of AML. Conclusion: In this case study, we diagnosed therapy-related acute myeloid leukemia. Of note, the theory of a common stem cell malignancy and the coexistence of two malignant clones seem not to be applied in the presented case. This is because an acute myeloid leukemia clone was not seen in her bone marrow, nor was peripheral blood evidenced at the time of her diagnosis of chronic lymphocytic leukemia.