Efficacy and safety of BT595 (10% human intravenous immunoglobulin) in adult patients with chronic immune thrombocytopenia.

Purpose: This trial investigated the efficacy and safety of the new 10% human intravenous immunoglobulin (IVIg) BT595 (Yimmugo®).

Methods: Adult patients with chronic immune thrombocytopenia (ITP) received a total dose of 2 g/kg body weight (bw) IVIg either over 2 or 5 days.

Results: Response as defined by the European Medicines Agency (EMA) was achieved in 18 of 34 patients (52.

Application of Rotational Thromboelastometry in Patients with Acute Promyelocytic Leukemia.

Introduction: Hemorrhagic early death (HED) remains a major cause of treatment failure among patients with acute promyelocytic leukemia (APL). We aimed to investigate the prognostic potential of rotational thromboelastometry (ROTEM) for bleeding in patients with APL.

Materials And Methods: 31 newly-diagnosed APL patients (median age of 40 years; 14 female/17 male) that underwent treatment at the Clinic of Hematology UCCS from 2016-2020 with all-trans retinoic acid and anthracyclines were recruited.

Rotational thromboelastometry (ROTEM) profiling of COVID-19 patients.

We evaluated coagulation abnormalities via traditional tests and rotational thromboelastometry (ROTEM) in a group of 94 patients with confirmed SARS-CoV-2 infection and different severity of pneumonia (34 moderate, 25 severe, 35 critical) with the hypothesis that ROTEM parameters differed by coronavirus disease 2019 (COVID-19) severity. Shorter than normal clotting time (CT) and higher than normal maximum clot firmness (MCF) in extrinsic rotational thromboelastometry (EXTEM) and fibrinogen rotational thromboelastometry (FIBTEM), shorter than normal EXTEM clot formation time (CFT), and higher than normal α-angle were classified as markers of hypercoagulable state. Increment in the number of patients with ≥2 hypercoagulable parameters, higher EXTEM ( = .

Peripheral neural response and sex hormones in type 1 Gaucher disease.

Background: In a rare Gaucher disease, reduced activity of lysosomal b-glucocerebrosidase incompletely blocks glucosphingolipid catabolism. Accumulation of the unhydrolyzed substrate glucosylceramide within lysosomes results in progressive, multisystem Gaucher disease, classified into three types. Both parkinsonism and peripheral neuropathy are observed in cases of putative non-neuronopathic type 1 disease.

Mucormycosis of the paranasal sinuses in a patient with acute myeloid leukemia.

Introduction: Invasive fungal infection is among the leading causes of morbidity, mortality, and economic burden for patients with acute leukemia after induction of chemotherapy. In the past few decades, the incidence of invasive fungal infection has increased dramatically. Its management has been further complicated by the increasing frequency of infection by non-Aspergillus molds (e.

Peripartum cardiomyopathy in a patient treated for acute myeloid leukemia.

Introduction: Peripartum cardiomyopathy usually presents with systolic heart failure during the last months of pregnancy and up to five months postpartum. The disease is rare and can be fatal.

Case Outline: We report a 30-year-old female who was diagnosed with acute myeloid leukemia, with maturation and cytogenetic finding of t(8;21)(q22;q22),del(9)(q22) in January 2004.

Lymphoproliferative neoplasms and renal cell carcinoma of clear cell type--Where is the link?

Introduction: The etiology of higher than expected occurrence of lymphoproliferative neoplasms (LPN) and renal cell carcinoma (RCC) in the same patient has not yet been clarified. Several explanations for this co-occurrence have been postulated: prior cytotoxic treatment, viral infections, immunomodulatory effects of tumor itself and shared genetic and/or environmental factors.

Case Report: Medical records of 680 consecutive patients with LPN and 570 consecutive patients with RCC diagnosed between January 1997 and December 2011 in two centers were retrospectively analyzed.

[Favorable Outcome of Hepatosplenic Candidiasis in a Patient with Acute Leukemia].

Introduction: Acute leukemias treatment requires strong chemotherapy. Patients that develop bone marrow aplasia become immunocompromised, thus becoming liable to bacterial and fungal infections. Fungal infections caused by Candida are frequent.

Thrombotic events in acute promyelocytic leukemia.

Introduction: Thrombotic events (TE) appear to be more common in acute promyelocytic leukemia (APL) than in other acute leukemias, with reported prevalence ranging from 2 to 10-15%.

Materials And Methods: We retrospectively analyzed the data on TE appearance in 63 APL patients.

Results: TE occured in 13 (20.

Complex haemostatic abnormalities as a cause of bleeding after neurosurgery in a patient with Gaucher disease.

We report a treatment-naïve patient with Gaucher disease (GD) who experienced repeated bleeding after three neurosurgeries for a brain tumour, identified as an oligoastrocytoma. The patient had normal values on basic haemostatic tests: prothrombin time, 75-105%; activated partial thromboplastin time, 30.3-34 s; and mild thrombocytopaenia, 96-115 × 10(9 )cells/l.

Acquired von Willebrand syndrome in patients with Gaucher disease.

Although various coagulation abnormalities occur in patients with Gaucher disease (GD), von Willebrand factor (vWF) deficiency has rarely been reported. A retrospective review of six treatment naïve cases with GD and concomitant vWF deficiency over a 12-year-period in a single center is presented. All patients had a personal history of prior hemorrhages.

Complex patterns of chromosome 11 aberrations in myeloid malignancies target CBL, MLL, DDB1 and LMO2.

Exome sequencing of primary tumors identifies complex somatic mutation patterns. Assignment of relevance of individual somatic mutations is difficult and poses the next challenge for interpretation of next generation sequencing data. Here we present an approach how exome sequencing in combination with SNP microarray data may identify targets of chromosomal aberrations in myeloid malignancies.

International Society of Thrombosis and Hemostasis Scoring System for disseminated intravascular coagulation ≥ 6: a new predictor of hemorrhagic early death in acute promyelocytic leukemia.

High-hemorrhagic early death (ED) rate is a major impediment in the managing of acute promyelocytic leukemia (APL). In our group of 56 newly diagnosed APL patients, ED occurred in 12 subjects, due to endocranial bleeding (8/12), differentiation syndrome (2/12), or infection (2/12). Predictors of hemorrhagic ED were as follows: white blood cells count ≥ 20 × 10(9)/L (P = 0.

Is there a "gold" standard treatment for patients with isolated myeloid sarcoma?

Isolated myeloid sarcoma is an extramedullary tumor of immature myeloid cells defined by the absence of leukemia history, myelodisplastic syndrome, or myeloproliferative neoplasma with a negative bone marrow biopsy. Myeloid sarcoma is a very rare condition, and few cases have been reported. We reviewed data of 12 patients with isolated myeloid sarcoma managed at a single center to determine the possible prognostic factors affecting patient survival, such as age, sex, type, localization, and treatment options.

Clinical significance of genetic aberrations in secondary acute myeloid leukemia.

The study aimed to identify genetic lesions associated with secondary acute myeloid leukemia (sAML) in comparison with AML arising de novo (dnAML) and assess their impact on patients' overall survival (OS). High-resolution genotyping and loss of heterozygosity mapping was performed on DNA samples from 86 sAML and 117 dnAML patients, using Affymetrix Genome-Wide Human SNP 6.0 arrays.

Concomitant aberrant methylation of p15 and MGMT genes in acute myeloid leukemia: association with a particular immunophenotype of blast cells.

In this study, methylation-specific polymerase chain reaction (MS-PCR) was used to define the methylation status of the target promoter sequences of p15 and MGMT genes in the group of 21 adult patients with acute myeloid leukemia (AML). The incidence of aberrant hypermethylation of p15 gene (71 %) was higher comparing to MGMT gene (33 %), whereas concomitant methylation of both genes had 24 % of the patients. Although the incidence of cytogenetic abnormalities between the groups with a different methylation status of p15 and/or MGMT genes was not significantly different, we observed general trend of clustering of abnormalities with adverse prognosis into groups with concomitant hypermethylation of both genes and only p15 gene.

Induction chemotherapy versus palliative treatment for acute myeloid leukemia in a consecutive cohort of elderly patients.

A retrospective survey of 210 consecutive patients aged ≥ 65 years (median age 69 years, range 65-88 years) with acute myeloid leukemia (AML) diagnosed at a single center over a 6-year period (January 2001 to December 2006) is presented. De novo AML was diagnosed in 179 (85.2 %) patients and 31 (14.

[Systemic mastocytosis: case report with literature review].

Introduction: Mastocytosis is a clonal neoplastic disorder of the mast cells. The clinical signs and symptoms of mastocytosis are heterogeneous ranging from indolent disease with a long-term survival to a highly aggressive neoplasm with survival of about 6 months. Systemic mastocytosis (SM) is characterized by mastocyte infiltration of one or more organs, with or without skin involvment.

Therapy-related acute leukemia in two patients with multiple sclerosis treated with Mitoxantrone.

Two cases of therapy-related acute leukemia (TRAL) after the use of Mitoxantrone for the treatment of secondary progressive multiple sclerosis (MS) are reported. They were extracted from the group of 42 consecutive patients with TRAL diagnosed and treated in single centre between 2000-2010. They were the only two with MS and the only two treated with Mitoxantrone.

Prognostic factors for therapy-related acute myeloid leukaemia (t-AML)--a single centre experience.

Prognostic parameters for treatment outcome in 42 consecutive patients with t-AML diagnosed and treated in a single centre between 2000-2010 (mean age: 56.07 years, range: 23-84; 30 females) were evaluated retrospectively/prospectively. Antecedent malignancy occurred in 37 patients (88.

Favourable prognostic factors in therapy related acute myeloid leukaemia.

Introduction: Therapy related acute myeloid leukaemia (t-AML) is a distinct clinical entity recognized by the World Health Organization classification occurring after chemotherapy and/or radiation treatment administered for a previous disease. T-AML is characterised by pancytopenia, three-lineage myelodysplasia, high frequency of unfavourable cytogenetics and short survival.

Objective: The aim of this study was to analyse clinical, cytogenetic, and cytological characteristics of t-AML and their impact on survival.

Haemostatic abnormalities in treatment-naïve patients with Type 1 Gaucher's disease.

There is a paucity of data on the effects of enzyme replacement therapy (ERT) on the coagulation abnormalities and platelet function of patients with Gaucher's disease (GDPs) and much of this data are controversial. This study investigates the haemostatic parameters in treatment-naïve GDPs and the effects of ERT. 31 Serbian treatment-naïve type 1 GDPs (M/F 17/14; median age 49 years, splenectomized 9/31) were studied.