Different clinical courses with the same findings: two cases of paroxysmal nocturnal hemoglobinuria presenting with thrombocytopenia

Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal stem cell disease that manifests with chronic intravascular hemolysis, thrombosis, and bone marrow failure. Various degrees of cytopenias accompany the disease. Although laboratory and clinical findings are similar, the disease may show different courses and require different treatments. Herein, we report two different courses of PNH with similar clinical and laboratory findings

A study of the correlation between the serum Latexin levels and the mTORC subunits Raptor and Rictor in the molecular pathogenesis of chronic lymphocytic leukemia

Hematopoietic stem cells (HSCs) ensure the lifelong production of blood cells throughout a lifetime. Latexin (Lxn) is thought to have a tumor suppressor role and endogenously down-regulate the number of HSCs via increased apoptosis. Therewithal, Raptor, and Rictor are components of the mammalian target of rapamycin complex-1, and 2 (mTORC), which are the regulatory structures for cell growth. However, Lxn, Raptor, and Rictor-associated molecular mechanisms underlying leukemia-induced HSCs proliferation are largely unknown. Nowadays, chronic lymphocytic leukemia (CLL) remains the most common leukemia type in adults. Therefore, we investigated the serum levels of Lxn, Raptor, and Rictor in CLL patients. We randomized 40 patients with newly diagnosed, untreated CLL. Serum levels of Lxn, Raptor, and Rictor were examined using ELISA assay. The results showed that serum Lxn levels reduced in patients with CLL. Moreover, the Rictor level increased in association with the up-regulation of leukocytosis. Although there was a tendency for an increase of the Raptor levels, the differences did not reach statistical significance. The up-regulated Raptor and Rictor levels in CLL suggested that it was associated with cancer pathogenesis. However, decreased Lxn levels raised the question of whether the disease is secondary to epigenetic features or if it is caused by pathology related to Lxn. The negative correlation between Lxn and Raptor/Rictor levels can provide new methods for the treatment of CLL, which are likely to increase the quality of life and improve the prognosis of the disease. In conclusion, further clinical studies are needed to elucidate the role of Lxn and Raptor/Rictor with the newly defined molecular properties in hematological malignancies and the clinical implications of their use. [Med-Science 2021; 10(2.000): 511-5

PAROXYSMAL NOCTURNAL HEMOGLOBINURIA PRESENTING WITH THROMBOCYTOPENIA: TWO PESG CASES

8th International Eurasian Hematology Oncology Congress (EHOC) -- OCT 18-21, 2017 -- Istanbul, TURKEYWOS: 00041674260013

Gaucher disease in an adult: A rare cause of hepatosplenomegaly in adults

WOS: 000433094100012PubMed ID: 2960743

Prevention of spontaneous combustion in coal drifts using a lining material: a case study of the Tuncbilek Omerler underground mine, Turkey

Synopsi

Eculizumab experience in an adult patient with atypical hemolytic uremic syndrome

Atypical hemolytic-uremic syndrome is a disease characterized by nonimmune hemolytic anemia, thrombocytopenia, and renal failure. In this study, we present a case of a patient with atypical hemolytic-uremic syndrome treated successfully with eculizumab. A 20-year-old female was admitted with clinical signs of atypical hemolytic syndrome. The laboratory findings were as follows: hemoglobin 9.2 g/dL, platelet count 18 × 103/μL, creatinine 4.69 mg/dL, schistocytes were in peripheral blood smear, lactate dehydrogenase 2080 U/L, and emergency plasmapheresis procedure with fresh frozen plasma were initiated. The patient was anuric within 12 h of her admittance. ADAMTS13 activity was normal. Her mother’s cousin developed acute rejection immediately after receiving a renal transplant and died two months later. As she did not respond to the treatment and considering her family history, eculizumab was initiated which resulted in platelet counts starting to rise on day 5, and the patient no longer needed dialysis after 22 days

Acute Monoblastic Leukemia Presenting with Multiple Granulocytic Sarcoma Nodules

WOS: 000398059500030PubMed ID: 2709472

Monoballism Associated with Newly Onset Ketotic Hyperglycemia

Movement disorders as the initial symptoms of diabetes mellitus are rare. Here, we describe one of these rare manifestations of primary diabetes: a case of newly diagnosed diabetes mellitus in an old-age female patient with transient monoballismus during an episode of ketotic hyperglycemia