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Sabina ZURAC

Latest posts by Sabina ZURAC (see all)

  • A case of Sweet’s syndrome secondary to myelodysplastic syndrome – diagnostic and treatment challenges - July 6, 2016
  • 2016 WHO Clinical Molecular and Pathological Criteria for Classification and Staging of Myeloproliferative Neoplasms (MPN) Caused by MPN Driver Mutations in the JAK2, MPL and CALR Genes in the Context of New 2016 WHO Classification: Prognostic and Therapeutic Implications - March 31, 2016

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A case of Sweet’s syndrome secondary to myelodysplastic syndrome – diagnostic and treatment challenges

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MÆDICA - a Journal of Clinical Medicine | Vol. 11, nr. 2, 2016 CNCSIS - CMR - B+ OBBCSSR

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A case of Sweet’s syndrome secondary to myelodysplastic syndrome – diagnostic and treatment challenges

Doinita SFRIJAN, Simina-Maria VISAN, Sabina ZURAC, Bianca DIACONU and Cristian SCURTU

ABSTRACT

Sweet’s Syndrome also knows as acute febrile neutrophilic dermatosis, is a rare skin’s condition, that can occur either idiopathic or secondary. In the case of the latter, the syndrome can develop after certain malignancies (paraneoplastic syndrome), because of exposure to some medication or post infectious. It is more frequent in women aged between 30 and 50 years, but concerning children, the disorder is extremely rare (8% of the total number of cases), having equal sex ratio distribution. We present the case of an 11 year old male, diagnosed with systemic form of SS associated with Myelodysplastic Syndrome. The onset of the hematological condition seemingly occurred at the age of 5, when the diagnosis of chronic immune thrombocytopenic purpura was established. The treatment included repeated cortisone administrations, followed by a splenectomy procedure. Admitted in our Oncopaediatric department in December 2012, the child is given the diagnosis of MS, to which severe systemical manifestations of SS were added, with partial treatment response (cortisone, cyclosporine, dapsone, indomethacin). An allogeneic bone marrow transplant was conducted at Fundeni Institute (February 2015) when the SS remission occurred, but the progression was fatal, the child developing graft-versus-host disease.

Keywords: Sweet’s Syndrome, Myelodysplastic Syndrome, Immune Thrombocytopenic Purpura, child

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2016 WHO Clinical Molecular and Pathological Criteria for Classification and Staging of Myeloproliferative Neoplasms (MPN) Caused by MPN Driver Mutations in the JAK2, MPL and CALR Genes in the Context of New 2016 WHO Classification: Prognostic and Therapeutic Implications

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MÆDICA - a Journal of Clinical Medicine | Vol. 11, nr. 1, 2016 CNCSIS - CMR - B+ OBBCSSR

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2016 WHO Clinical Molecular and Pathological Criteria for Classification and Staging of Myeloproliferative Neoplasms (MPN) Caused by MPN Driver Mutations in the JAK2, MPL and CALR Genes in the Context of New 2016 WHO Classification: Prognostic and Therapeutic Implications

Jan Jacques MICHIELS, Mihaela TEVET, Adrian TRIFA, Emilia NICULESCU-MIZIL, Anca LUPU, Ana-Maria VLADAREANU, Horia BUMBEA, Anca ILEA, Camelia DOBREA, Daniela GEORGESCU, Oana PATRINOIU, Mihaela POPESCU, Meilin MURAT, Cornel DRAGAN, Felicia MIHAI, Sabina ZURAC, Silvana ANGELESCU, Anamaria IOVA, Alina POPA, Rodica GOGULESCU and Viola POPOV

ABSTRACT

The 2016 WHO-CMP classification proposal defines a broad spectrum of JAK2 V617F mutated MPN phenotypes: normocellular ET, hypercellular ET due to increased erythropoiesis (prodromal PV), hypercellular ET with megakaryocytic-granulocytic myeloproliferation and splenomegaly (EMGM or masked PV), erythrocythemic PV, early and overt classical PV, advanced PV with MF and post-PV MF. ET heterozygous for the JAK2 V617F mutation is associated with low JAK2 mutation load and normal life expectance. PV patients are hetero-homozygous versus homozygous for the JAK2 V617F mutation in their early versus advanced stages with increasing JAK2 mutation load from less than 50% to 100% and increase of MPN disease burden during life long follow-up in terms of symptomatic splenomegaly, constitutional symptoms, bone marrow hypercellularity and secondary MF. Pretreatment bone marrow biopsy in prefibrotic MPNs is of diagnostic and prognostic importance. JAK2 exon 12 mutated MPN is a distinct benign early stage PV. CALR mutated hypercellular thrombocythemia show distinct PMGM bone marrow characteristics of clustered larged immature dysmorphic megakaryocytes with bulky (bulbous) hyperchromatic nuclei, which are not seen in JAK2 mutated ET and PV. MPL mutated normocellular thrombocythemia is featured by clustered giant megakaryocytes with hyperlobulated stag-horn-like nuclei without features of PV in blood and bone marrow. Myeloproliferative disease burden in each of the JAK2, CALR and MPL MPNs is best reflected by the degree of anemia, splenomegaly, mutation allele burden, bone marrow cellularity and myelofibrosis.

Keywords: Myeloproliferative neoplasms; Essential thrombocythemia; Polycythemia vera; Primary megakaryocytic granulocytic myeloproliferation; Myelofibrosis; JAK2V617F mutation; MPL515 mutation; Calreticulin mutation; JAK2 wild type; Bone marrow pathology

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