Andrzej Mital

A case of mast cell leukaemia with exon 9 KIT mutation and good response to imatinib

Background:u2002 Mastocytosis is a myeloproliferative neoplasm characterized by the excessive proliferation of mast cells. Mast cell leukaemia (MCL), the aggressive form of this disease, requires cytoreductive therapy, such as cladribine, interferon‐alpha‐2b and, most recently, tyrosine kinase inhibitors – dasatinib or imatinib.

Erythrophagocytosis by neutrophils – a rare morphological phenomenon resulting in acquired haemolytic anaemia?

Erythrophagocytosis by neutrophils is a rare morphological phenomenon described in patients with clonal malignancies of haematopoiesis with myelodysplasia and in some haemolytic conditions including paroxysmal cold haemoglobinuria, haemolysis caused by snake‐bite, sickle cell anaemia and other defects of red cells. We describe a female patient who presented with acquired haemolytic anaemia. Erythrophagocytosis was found in around 35% of neutrophils of the peripheral blood. A similar picture was seen in the bone marrow, but with additional erythrophagocytosis by macrophages. These two processes were considered as the main causes of anaemia, but the first one seemed to be predominant. Malignancies, autoimmunisation disorders and infections were excluded. Immunosuppressive therapy with corticosteroids was implemented, but had to be stopped because of side effects. Long‐term normalization of peripheral blood morphology was achieved after splenectomy. Splenectomy may be considered a therapeutic option for patients with diagnosed neutrophil erythrophagocytic hyperactivity. Therapy with corticosteroids is also possible, but the long‐term effects remain unknown.

Factors predisposing to acquired von Willebrand syndrome during the course of polycythemia vera - retrospective analysis of 142 consecutive cases.

INTRODUCTIONnThe aim of this study was to verify if PV patients with and without avWS differ in terms of their baseline clinical parameters.nnnMATERIAL AND METHODSnThe study included 142 consecutive patients with PV. avWS was diagnosed on the basis of abnormally low levels of von Willebrand factor and other routine tests. Patients with and without concomitant avWS were compared in terms of their demographic characteristics, present and past medical histories and laboratory parameters.nnnRESULTSnConcomitant avWS was found in 17 PV patients (12.0%). Individuals with avWS have been diagnosed with PV at significantly younger age than those without, and significantly less often were in remission at the time of testing for bleeding disorders. Most of them (58.8%) presented with typical signs of bleeding disorder. Moreover, they showed significantly higher erythrocyte, leukocyte and platelet counts, abnormalities of coagulation profile corresponding to defects of primary hemostasis and abnormal values of all parameters used in the routine diagnosis of avWS.nnnCONCLUSIONSnEven every tenth patient with PV may develop avWS. Young age at diagnosis of PV and poor response to previous treatment of this condition are potential risk factors of avWS that should be considered during history taking. Sings of bleeding disorder observed in a person with PV necessitate evaluation for avWS.

Demographic, Hematologic, and Clinical Features of Myelodysplastic Syndrome Patients: Results from the First Polish Myelodysplastic Syndrome Registry.

Epidemiological studies on myelodysplastic syndromes (MDS) in Middle-Eastern Europe are scarce. No data about the demographic, clinical, and laboratory features of Polish MDS patients have been published. The aim of this study was to assess the epidemiological data and toxic exposure of Polish MDS patients and their association with hematological parameters and clinical outcomes. For 15 months, 966 living MDS patients were enrolled at 24 centers (12 university and 12 community hospitals). Follow-up was conducted for the next 55 months. The percentage of patients older than 80 years (16%) was between the values for Eastern and Western countries. In patients younger than 55 years, a female predominance was observed (male/female ratio 0.70:1 vs. 1.29:1; p < 0.001). Female patients had higher platelet counts (160 × 109/l vs. 111 × 109/l; p < 0.001). Patients exposed to chemicals were younger than patients without such exposure; their median age at MDS diagnosis was 66 vs. 70 years (p = 0.037). Smokers had significantly lower hemoglobin concentrations (8.6 vs. 9.1 g/dl; p = 0.032) and lower platelet counts (99 × 109/l vs. 137 × 109/l; p < 0.001) than nonsmokers. We provide the first description of the characteristics of Polish MDS patients. Females predominated in the group aged <60 years and they had higher platelet counts. The course of the disease is affected by toxic exposure and smoking.

Polish experience of lenalidomide in the treatment of lower risk myelodysplastic syndrome with isolated del(5q).

BackgroundLenalidomide has been approved for the treatment of lower-risk myelodysplastic syndrome (MDS) with 5q deletion (del(5q)). We present for the first time a retrospective analysis of low-risk MDS with isolated del5q treated with lenalidomide, outside the clinical trials.Methods36 red blood cell (RBC) transfusion-dependent patients have been included in the study. Patients received lenalidomide 10xa0mg/day on days 1–21 of 28-day cycles.Results91.7xa0% of patients responded to lenalidomide treatment: 72.2xa0% achieved erythroid response, 19.4xa0% achieved minor erythroid response and 8.4xa0% of patients did not respond to treatment. Response depended on number of previous treatment lines (pu2009=u20090.0101), International Prognostic System Score (IPSS; pu2009=u20090.0067) and RBC transfusion frequency (pu2009=u20090.0139). Median duration of response was 16xa0months (range 6–60 months). Treatment was well tolerated. We observed hematological toxicity (grade 3 and 4): neutropenia in 16 (44.4xa0%) patients and thrombocytopenia in 9 (25xa0%) patients. Two patients (5.5xa0%) progressed to high-risk MDS and two subsequent progressed to acute myeloid leukemia. A Kaplan-Meier estimate for overall survival at 5xa0years in the study group was 79.0u2009±u20098.8xa0%.ConclusionsLenalidomide in this group of patients was beneficial for the treatment of RBC transfusion-dependency with well-known safety profile.

Are Myelodysplastic Syndromes Underdiagnosed in Poland? A Report by the Polish Adult Leukaemia Group

The epidemiology of myelodysplastic syndromes (MDS) differs among countries. Here, we present the first epidemiological indices determined for Poland.

Assessment of platelet function in endogenous hypercortisolism.

INTRODUCTIONnIt is commonly known that glucocorticoids exert a significant effect on haemostasis. Studies that have analysed the plasmatic coagulation system and fibrinolysis parameters in hypercortisolaemic patients are abundant. Platelet function, which plays a vital role in primary haemostasis, is much less clear in this context. We aimed at assessing platelet function in endogenous hypercortisolaemic patients.nnnMATERIAL AND METHODSnTwenty-five hypercortisolaemic patients were included in the study. Twelve of them were diagnosed with overt Cushings syndrome (OCS) and 13 had subclinical Cushings syndrome (SCS). Thirty healthy volunteers comprised the control group. In all subjects platelet function parameters were examined: ADP- and collagen-induced platelet aggregation (ADP-IPA and Col-IPA, respectively), IMPACT R (expressed as percentage of surface covered (SC) by platelets and average size (AS) of the adhering particles in μm2), as well as closure time (CT) after platelet activation with agonists: ADP and Col or Col and epinephrine (EPI). The statistical significance level was set at 0.05.nnnRESULTSnThere was no significant difference in mean values of ADP-IPA, Col-IPA, Col/Epi CT, Col/ADP CT, SC, and AS between hypercortisolaemic subjects and controls. No statistically significant differences in means of examined parameters were found between overt and subclinical Cushings syndrome patients. Furthermore, no statistically significant relationships were found between these parameters and hormonal indicators of hypercortisolism: 24-hour urinary cortisol excretion, morning and evening serum cortisol level, and overnight-test cortisol concentration.nnnCONCLUSIONSnIn hypercortisolaemic patients no primary haemostasis disorders are present, as reflected by platelet adhesion and ADP- and collagen-induced aggregation measurements.

Immune thrombocytopenia in patients with chronic lymphocytic leukemia treated with cladribine-based regiments or chlorambucil – follow-up of PALG-CLL randomized trials

The relationship between treatments of chronic lymphocytic leukemia (CLL) with cladribine (2‐CdA) or chlorambucil and immune thrombocytopenia (IT) has not been yet determined.

Hemostatic Disorders in Hormonally Active Pituitary Tumors

Endocrinopathies encompass heterogeneous diseases that can lead to hemostasis disorders at various stages over their clinical course. Normal hemostasis requires an equilibrium between the processes of coagulation and fibrinolysis, which depend on multiple activators and inhibitors. To date, the influence of various hormonal disorders on the hemostatic system has been assessed many times. The aim of this review was to analyze hemostasis abnormalities that occur in patients with hormonally active pituitary tumors: corticotropinoma, somatotropinoma, prolactinoma, gonadotropinoma and thyrotropinoma. Authors discuss studies that examined coagulation and hemostasis parameters among patients with these tumors, as well as analyze antithrombotic prophylaxis approach for endogenous hypercortisolemia subjects in particular.

Necrotizing enterocolitis in acute lymphoblastic leukemia patients : Department experience

Necrotizing enterocolitis is a severe complication of high-dose chemotherapy in patients with hematopoietic malignancies. Some patients with severe mucositis may develop necrosis of the intestine, leading to typhlitis and perforation of the intestinal tract. The patients have few obvious symptoms; thus, early diagnosis and prompt treatment of intestinal necrosis or bowel perforation seem critical for a good overall treatment outcome. We present 5 cases of intraabdominal complications in patients with acute lymphoblastic leukemia. All patients who underwent surgery survived the postoperative period. We also present a suggested algorithm for treatment of acute abdomen complications in leukemic patients.