Are myelodysplastic syndromes underdiagnosed in Poland? A report by the Polish Adult Leukaemia Group.

OBJECTIVES: The epidemiology of myelodysplastic syndromes (MDS) differs among countries. Here, we present the first epidemiological indices determined for Poland. METHODS: Twenty-one haematological centres participated in the study. Patients diagnosed with MDS and acute myeloid leukaemia (AML) with 20-29% blasts were enrolled. Data collection was conducted for strictly predefined period. RESULTS: The overall crude incidence rate for all MDS subtypes was 1.95 (95% CI, 1.81-2.09) per 100 000 person-years: 2.46 (95% CI, 2.24-2.69) for males and 1.47 (95% CI, 1.31-1.65) for females; after excluding AML cases, the indices were as follows: 2.35 (95% CI, 2.08-2.66) for males and 1.27 (95% CI, 1.08-1.5) for females. Prevalence rate was 6.2 per 100 000 persons (95% CI, 5.96-6.45), that is 6.86 (95% CI, 6.49-7.24) for males and 5.58 (95% CI, 5.26-5.92) for females. Both incidence and prevalence increased with increasing age. The most frequently diagnosed MDS subtype was refractory cytopenia with multilineage dysplasia (RCMD), responsible for 30.3% of all newly diagnosed MDSs. CONCLUSIONS: RCMD is the most frequent MDS subtype in Poland. Incidence and prevalence indices are lower than those reported for other populations, which probably results from inadequate diagnosis of potential cases of this disease.

[The role of fibrocytes in pulmonary fibrosis]. / Rola fibrocytów w procesach wlóknienia pluc.

Fibrocytes are bone-marrow derived mesenchymal progenitor cells. They express typical markers of leukocytes, hematopoietic stem cells and fibroblasts. They play a pivotal role in the tissue remodeling and fibrosis in both physiologic and pathologic settings. Fibrocytes are unique in that they are capable of differentiating into fibroblasts and myofibroblasts, as well as adipocytes. Circulating fibrocytes may play a role in pulmonary fibrosis and clinical outcomes. Recent data obtained from the clinical setting suggest that high numbers of circulating fibrocytes correlate with pulmonary function test parameters and disease activity in patients with different interstitial lung diseases. A greater understanding of the immunologic mediator that influence fibrocyte biology suggest new opportunities for therapeutic manipulation of these cells in fibrogenesis.

The morphology of myeloma cells changes with progression of the disease.

AIM OF THE STUDY: Multiple myeloma is a heterogeneous entity with variable course. Plasma cells found in bone marrow smears are characterised by extremely high diversity of morphology. We have attempted to determine whether the morphological characteristics of myeloma cells vary with the natural course of the disease. We investigated the incidence of selected morphological features and planimetric parameters of myeloma cells present in bone marrow smears. MATERIAL AND METHODS: Material collected from 103 patients was evaluated at diagnosis and then during relapse. It was found that in the same patients, plasma cell morphology changes in the course of the disease: cell surface, nucleus surface, tumour cell anisocytosis and nuclear-cytoplasmic ratio increase significantly. RESULTS: The results suggest that some morphological features are more common in clinically advanced disease. These include the number of nucleoli, the number of myeloma cells with irregular nuclei, and larger nuclei. Using the classification systems according to Greipp and Goasguen, we have noted changes in morphological pattern of myeloma cells in some patients with progressive multiple myeloma. This was associated with the appearance of a cell clone characterised by a set of traits indicating a low degree of maturity. CONCLUSIONS: We did not find that the type and intensity of cytostatic therapy significantly affect the morphology of plasma cells. Therefore, we suggest that some changes are due to natural, expansive course of the disease.

[Essential thrombocythemia as critical stroke risk factor--case report]. / Nadplytkowosc samoistna istotnym czynnikiem ryzyka udaru mózgowego--opis przypadku.

One of the haematological causes of stroke is essential thrombocythemia (ET). It is one of the proliferative syndromes of the haematopoietic system. Patients with ET have an increased risk of thrombosis and/or haemorrhage of veins and arteries. The patient aged 58 had a history of two stroke incidents within two month despite the treatment with acenocumarole for chronic atrial fibrillation. The clinical diagnostic procedure revealed an increased platelet count was 668 000/ml, and these cerebrovascular events were the first manifestation of ET Antithrombotic drugs were not effective in the secondary prevention of stroke while antiplatelets prevented this patient from further ischemic events for 12 months.

Mobilization of Hematopoietic Stem Cells for Hematopoietic Cells Autologous Transplantation with Use of Plerixafor.

BACKGROUND To increase the number of circulating hematopoietic stem cells (HSC) in the blood, mobilization treatments are currently being used. G-CSF and G-CSF plus chemotherapy are the most common methods of hematopoietic stem cells separation used in Poland. MATERIAL AND METHODS We observed patients who failed an effective hematopoietic stem cell mobilization with G-CSF or with G-CSF plus chemotherapy. The separation was considered unsuccessful if within a period of 4 consecutive days of separation, the number of obtained CD 34+ cells was lower than 2.0×10^6/kg of bodyweight. The study involved 32 patients whose CD34+ cells were collected and the collection for autologous transplantation failed. The study included 20 men and 12 women. Among all 32 patients, 28 had multiple myeloma, 3 had DLBCL lymphoma, and 1 had Hodgkin's disease. RESULTS Separation was unsuccessful in only 3 patients; the remaining 29 achieved an average of 4.83×10^6 CD34+ cells per kg of bodyweight. We conclude that plerixafor is an important tool in obtaining sufficient quantities of cells for hematopoietic stem cells separation. CONCLUSIONS The use of plerixafor is a sufficient and safe option for stem cells mobilization in autologous transplantations.

Acute Phase Proteins for Monitoring Hematopoietic Recovery After Early Engrafting of CD34+ Peripheral-Blood-Stem-Cells for Autografting or Allografting in Patients with Malignant Diseases.

BACKGROUND Neutropenic fever (NF) is associated with delayed engraftment after peripheral blood stem cell transplantation (PBSCT). MATERIAL AND METHODS We followed the levels of acute-phase proteins (APPs) serially in 60 patients after peripheral blood stem-cell autograft (n=39) or peripheral blood stem-cell allograft (n=21) for hematologic malignancies and germinal tumors; we then examined the correlation of those levels with the presence of fever and with markers of engraftment. RESULTS Fever (present in 60% of patients) was associated with a highly statistically significant delay in reaching conventional engraftment targets (ANC >500/µL [0.5×10^9/L]; platelets >20,000/µL [20×10^9/L]; reticulocytes >20,000/µL [20×10^9/L]) (for all associations, p<0.001). Every 4th day for 24 days, we measured the APPs levels and the number of neutrophils (ANC), platelets (PL), and reticulocytes (RET) to reach the reference values of >0.5 G/L or >1.0 G/L for ANC, >20 G/L or >50 G/L for PL, and >20 G/L for RET, respectively. The presence of NF resulted in longer time to engraft hematopoietic stem cells with ANC, PL, and PET counts statistically significant (range 0.001-0.004). The median day range for NF patients was 21.22-26.89 versus 13.88-19.13 for no NF patients. CONCLUSIONS Our results provide additional information for monitoring hematopoietic engraftment in patients following PBSCT; the presence of NF can be tracked by serial measurements in serum of three investigated APPs throughout an early phase of hematopoietic recovery.

Randomized comparison of cladribine alone or in combination with cyclophosphamide, and cyclophosphamide, vincristine and prednisone in previously untreated low-grade B-cell non-Hodgkin lymphoma patients: final report of the Polish Lymphoma Research Group.

BACKGROUND: The objective of this study was to compare the efficacy of 3 regimens, cladribine alone, cladribine and cyclophosphamide combination, or cyclophosphamide, vincristine, and prednisone combination in previously untreated patients with low-grade B-cell non-Hodgkin lymphoma (LGNHL). METHODS: For this 3-arm, phase 3 study, 197 patients were randomly allocated to receive 6 monthly courses of cladribine alone, cladribine and cyclophosphamide combination, or cyclophosphamide, vincristine, and prednisone combination. Patients for whom all clinical data were available and 162 patients who completed scheduled chemotherapy were analyzed for the endpoints of this study. RESULTS: Compared with cyclophosphamide, vincristine, and prednisone combination regimen, cladribine alone or cladribine and cyclophosphamide combination induced higher probability of overall response (odds ratio [OR] = 4.0; 95% confidence interval [CI], 1.7-9,3; P = .002, and OR = 8.5; 95% CI, 3.2-22.7; P < .0001, respectively), complete remission (OR = 5.8; 95% CI, 1.8-18.5; P = .003; and OR = 14; 95% CI, 4.4-44; P < .0001, respectively), progression-free survival (log-rank test P < .0001), but not overall survival. After incorporating the International Prognostic Index in multivariate analysis, treatment with cladribine-containing regimens remained an independent prognostic factor for progression-free survival (chi(2) = 35.94; hazard ratio = 2.38; P < .0002). Incidences of infections were similar in the randomized groups, whereas cladribine and cyclophosphamide combination, but not cladribine alone, induced more frequent neutropenia, anemia, and thrombocytopenia compared with cyclophosphamide, vincristine, and prednisone combination (P < .05 for each). This resulted in a higher frequency of prolongation of intervals between cladribine and cyclophosphamide combination and cyclophosphamide, vincristine, and prednisone combination cycles (P < .05), but dose reductions due to hematological or other toxicity did not differ significantly in cladribine alone, cladribine and cyclophosphamide combination, and cyclophosphamide, vincristine, and prednisone combination groups. CONCLUSIONS: For patients with LGNHL, first-line cladribine alone or cladribine and cyclophosphamide combination regimens both provided similar treatment responses, acceptable toxicity, and better response rates than cyclophosphamide, vincristine, and prednisone combination.