Białaczka włochatokomórkowa oporna na standardową terapię analogiem puryn – opis przypadku i przegląd piśmiennictwa

Hairy cell leukemia (HCL) is a rare, chronic lymphoproliferative disorder. Currently, purine nucleoside analogs (PNA) constitute the first line treatment of HCL. Cladribine could induce long lasting remission in majority of patients with only a single cycle of therapy. In fact the relapsed patients could be treated successfully with cladribine too. Sometimes we observe the resistance to PNA. Rituximab and chemoimmunotherapy (rituximab plus cladribine) are effective in treatment of refractory HCL.We present a case of a 64-year-old man who was treated with rituximab after second progression of HCL. In March 2011, rituximab was given at a dose of 375 mg/m² i.v. once a week for eight weeks, with result of achievement of PR. During the last hospitalization in March 2013 the persistence of PR was confirmed.     

Małopłytkowość – wskazania do zastosowania cytokin płytkotwórczych

The most common cause of isolated thrombocytopenia is primary immune thrombocytopenia (ITP). For patients failing initial corticosteroid-based treatment and with refractory ITP post-splenectomy, thrombopoietin receptor agonists are indicated. Two of this thrombopoiesis-stimulating agents have been approved for use in ITP – eltrombopag, formulated for oral administration, once a day and romiplostim, which is administered weekly as a subcutaneous injection.     

Factors related to early termination of breast-feeding in an urban population

A prospective study of breast-feeding mothers was undertaken to determine the effect of formula samples and other hospital-related factors on success in breast-feeding. Of the 166 nursing mothers studied for 4 months postpartum, 83% breast-fed for 1 month, 73% for 10 weeks, and 58% for 4 months or longer. Breast-feeding duration was not affected by formula samples given at discharge from the hospital. Factors correlating significantly with improved breast-feeding rates include maternal age, maternal education, nonsmoking, previous breast-feeding, planned pregnancy, initiation of breast-feeding in the first 16 hours, and minimization of formula supplementation in the nursery. Partial breast-feeding (supplementing more than one bottle of formula per day, measured at 1 month postpartum) was associated with shorter breast-feeding duration. This latter effect was minimized by frequent nursing (seven or more times per day), despite formula supplementation.     

Topical interleukin-8 antibody attracts leukocytes in a piglet lavage model

Objective Acute respiratory distress syndrome (ARDS) in young infants is linked with a pulmonary inflammatory response part of which are increased interleukin-8 (IL-8) levels and migration of polymorphonuclear leukocytes (PMNL) into lung tissue. A topical application of an antibody against IL-8 might therefore decrease PMNL migration and improve lung function.Design Randomized, controlled, prospective animal study.Setting Research laboratory of a university childrens hospital.Subjects and interventions Anesthetized, mechanically ventilated newborn piglets (n=22) underwent repeated airway lavage to remove surfactant and to induce lung inflammation. Piglets then received either surfactant alone (S, n=8), or a topical antibody against IL-8 admixed to surfactant (S+IL-8, n=8), or an air bolus injection (control, n=6).Measurements and results After 6 h of mechanical ventilation following intervention, oxygenation [S 169±51 (SD) vs S+IL-8 139±61 mmHg] and lung function (compliance: S 1.3±0.4 vs S+IL-8 0.9±0.4 ml/cmH₂O/kg; extra-vascular lung-water: S 27±9 vs S+IL-8 52±28 ml/kg) were worse in the S+IL-8 group because reactive IL-8 production [S 810 (median, range 447–2323] vs S+IL-8 3485 (628–16180) pg/ml; P<0.05) with facilitated migration of PMNL into lung tissue occurred. Moreover, antibody application caused augmented chemotactic potency of IL-8 [linear regression of migrated PMNL and IL-8 levels: S r²=0.30 (P=ns) vs S+IL-8 r²=0.89 (P=0.0002)].Conclusion Topical anti-IL-8 treatment after lung injury increases IL-8 production, PMNL migration, and worsens lung function in our piglet lavage model. This effect is in contrast to current literature using pre-lung injury treatment protocols. Our data do not support anti-IL-8 treatment in young infants with ARDS.Financial support: supported by Hübner-Stiftung im Stifterverband, Essen; and Deutsche Forschungsgemeinschaft, Bonn, grant KR 1863/1-1     

Overexpression of sPRDM16 coupled with loss of p53 induces myeloid leukemias in mice

Transgenic expression of the abnormal products of acute myeloid leukemia-associated (AML-associated) primary chromosomal translocations in hematopoietic stem/progenitor cells initiates leukemogenesis in mice, yet additional mutations are needed for leukemia development. We report here aberrant expression of PR domain containing 16 (PRDM16) in AML cells with either translocations of 1p36 or normal karyotype. These carried, respectively, relatively high prevalence of mutations in the TP53 tumor suppressor gene and in the nucleophosmin (NPM) gene, which regulates p53. Two protein isoforms are expressed from PRDM16, which differ in the presence or absence of the PR domain. Overexpression of the short isoform, sPRDM16, in mouse bone marrow induced AML with full penetrance, but only in the absence of p53. The mouse leukemias were characterized by multilineage cellular abnormalities and megakaryocyte dysplasia, a common feature of human AMLs with 1p36 translocations or NPM mutations. Overexpression of sPRDM16 increased the pool of HSCs in vivo, and in vitro blocked myeloid differentiation and prolonged progenitor life span. Loss of p53 augmented the effects of sPRDM16 on stem cell number and induced immortalization of progenitors. Thus, overexpression of sPRDM16 induces abnormal growth of stem cells and progenitors and cooperates with disruption of the p53 pathway in the induction of myeloid leukemia.     

Local and diffuse spread of juvenile cerebellar astrocytomas in subarachnoid space. Light and electron microscopic study.

Based on 5 cases of juvenile astrocytomas the unusual behaviour of this type of tumor is described. It is expressed by local and/or diffuse spread of the neoplasms in the subarachnoid space of the brain hemispheres, cerebellum and spinal cord. The light and electron microscopic examinations have revealed the morphological features of well differentiated tumors of astrocytic origin and of fibrillary type which in a great number of cases have a favourable prognosis. The cause of break through into the subarachnoid space, despite its morphology which indicates on a high degree of differentiation of the tumors, is not known. The relation of primary to secondary infiltration of the subarachnoid space or of the leptomeninges is discussed.