Finger ischemia in a young lady: an unusual presentation of papillary fibroelastoma with intraventricular location.

An otherwise healthy 32-year-old woman suffered from finger ischemia. An echocardiogram and computed tomography scan revealed a mobile mass in the left ventricle that was attached to the anterior papillary muscle and did not involve the valve leaflets. The tumor was resected, and histopathology confirmed it to be a papillary fibroelastoma. Our case emphasizes the significance of a comprehensive diagnostic work-up for a peripheral ischemic lesion. This resulted in the discovery of an unusual intra-ventricular origin for a commonly benign tumor.

COVID-19 acute respiratory distress syndrome: can iloprost have a role for the treatment?

Pulmonary infection of 2019-nCoV can frequently induce acute respiratory distress syndrome (ARDS) with partial pressure of arterial oxygen/fraction of inspired oxygen ratio (pO2/FiO2) of less than 300 mmHg. Moreover, it can be complicated with cardiac injury or arrhythmia, microvascular and large-vessel thrombosis. We describe a case of a patient with COVID19-ARDS and concomitant critical ischemia of the limbs. Iloprost treatment, an analogue of a prostacyclin PGI2, was started for residual left forefoot ischemia after surgical thromboembolectomy. Unexpectedly, we documented improvement of respiratory performance and lung high resolution computed tomography (HRCT) showed significant regression of the diffuse pulmonary ground-glass opacity. The hypothetical mechanism is that iloprost can enhance perfusion preferentially to well-ventilated lung regions, reduce pressures of peripheral pulmonary vessels and induce reduction of lung interstitial edema. In addition, iloprost antithrombotic effect, endothelial damage repairing and neo-angiogenesis activity could play a relevant role.

Dark sputum: An atypical presentation of primary pulmonary malignant melanoma.

Primary melanoma of the lung is an extremely rare clinical entity. We found only 32 cases reported in literature, and in two of these multiple brain metastases were present. We describe a case of primary lung melanoma with brain and skin metastases that presented with an initial clinical diagnosis of pneumonia. A 55-year-old white man presented with cough productive of dark sputum and fever. A chest x-ray showed a right lung infiltration. After failure to respond to usual treatment for pneumonia, bronchoscopy examination and CT scan revealed a right pulmonary mass. The CT-guided biopsy confirmed a diagnosis of malignant melanoma. The primary lung origin of the tumor was demonstrated by the characteristic junctional pattern of melanoma cells. Further evaluation revealed metastases in the brain and in skin. Primary lung melanoma is an uncommon neoplasm that may be confused with more conventional types of lung cancer. Careful interpretation of histopathological information in correlation with all other clinical, laboratory and imaging studies may be needed to establish a diagnosis. Evaluation for metastases should include looking at the eyes, brain, skin. Due to the small number of cases reported in literature, there is no experience on the management and the prognosis of the disease.

Heat-shock protein-27, -70 and peroxiredoxin-II show molecular chaperone function in sickle red cells: Evidence from transgenic sickle cell mouse model.

Sickle cell disease (SCD) is an autosomal recessive genetic red cell disorder characterized by the production of a defective form of hemoglobin, hemoglobin-S, that is worldwide-distributed. The acute clinical manifestations of SCD are related to hemoglobin cyclic-polymerization and to the generation of rigid, dense red blood cells (RBCs). We studied RBCs membrane proteome from human sickle RBCs, fractioned according to density compared to normal RBCs. 2-DE followed by MS analysis was carried out. We identified 65 proteins differently expressed, divided into five major clusters according to their functions: (i) membrane-cytoskeleton proteins; (ii) metabolic enzymes; (iii) ubiquitin-proteasome-system; (iv) flotillins; (v) chaperones. HSP27, HSP70 and peroxiredoxin-II (Prx-II) showed the most relevant changes. They were differently recruited to sickle RBCs membrane in response to in vitro hypoxia. Potential markers were then validated in a transgenic-mouse model for SCD, the SAD mice, exposed to hypoxia mimicking acute SCD vaso-occlusive-crisis (VOCs); we found that HSP70 and HSP27 bound to RBCs membrane respectively after 12 h and 48 h of hypoxia, while Prx-II membrane binding was modulated during hypoxia. Our data indicate that HSP27 and HSP70 play a novel role as RBCs membrane protein protectors and as possibly new markers of severity of RBCs membrane damage during acute VOCs.

Acute hemolytic anemia with acanthocytosis associated with high-dose misoprostol for medical abortion.

We report a case of acute hemolytic anemia in a 21-year-old Nigerian woman after high-dose misoprostol (4 mg), used for medical abortion. The major causes of inherited or immune hemolytic anemia were excluded. The patient's peripheral blood smear showed acanthocytes and anisopoikilocytosis, which progressively disappeared in the days postingestion. We evaluated RBC features, and we observed reduced RBC Na+ and K+ content and abnormalities in membrane cation transport pathways and in Ca2+ activated K+ channel (Gardos channel), suggesting possible direct effects of misoprostol on RBCs. Although further studies need to be carried out, the present case suggests that high-dose misoprostol, a prostaglandin E1 analogue, severely affects RBC features and causes an acquired acute hemolytic anemia, which is self-limited when misoprostol is withdrawn.

Liver expression of hepcidin and other iron genes in two mouse models of beta-thalassemia.

BACKGROUND AND OBJECTIVES: Homozygous beta-thalassemia patients may develop iron overload even if untransfused, due to inappropriately high intestinal iron absorption. Reduction of hepcidin synthesis has been reported both in patients and in animal models. We have measured liver hepcidin and other iron gene transcripts in two different mouse models of beta-thalassemia at different ages. DESIGN AND METHODS: Mice Hbb(th/th), characterized by spontaneous homozygous deletion of the major b1 globin gene were studied at 2 and 8 months. Mice Hbb(th/3+), characterized by the heterozygous deletion of b1 and b2 globin genes were studied at 4 and 10 months. Hematologic data were obtained and iron overload estimated by Perls' staining of the liver. Expression of liver hepcidin, Tfr2, Hjv, Fpn and Hfe RNA was assessed by real-time polymerase chain reaction. Levels of serum cytokines (interleukin-6, IL-1beta, IL-10, granulocyte-macrophage colony-stimulating factor) levels were assayed by enzyme-linked immunosorbent assay. RESULTS: Hemoglobin, hematocrit and mean corpuscular volume were significantly reduced in both beta-thalassemia models, more significantly in Hbb(th/3+), which have the greater, age-dependent, iron overload. Hepcidin RNA was not increased despite iron overload in both strains. Fpn RNA was increased and Tfr2 was decreased in older animals. Inflammatory cytokine levels were striking variable and unrelated to hepcidin levels. INTERPRETATION AND CONCLUSIONS: Although anemia is reported to inhibit hepcidin expression, normal hepcidin synthesis was maintained in both thalassemic models studied. However, hepcidin levels were inappropriate for the body iron, especially in Hbb(th/3+) 10-month-old animals. As we previously reported in wild type mice after parenteral iron overload, Tfr2 is reduced and Fpn RNA increased in thalassemic mice. Inflammatory cytokines did not play a major role in increasing hepcidin levels or in modifying iron homeostasis in this study.