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INHERITED BONE MARROW FAILURE DISEASESOP 19
Ghosal hematodiaphyseal dysplasia (GHDD) diagnosis and treatment: case report
Gular MAMMADOVA1, Samira HASANOVA1, Konul BAGHIROVA1, Avesta ALLAHVERDIYEVA1, Narmin EYVAZOVA1, Adam NAJAFLI1, Kamala MAMMADOVA1, Afag NASIBOVA1, Valeh HUSEYNOV1,2
1 Azerbaijan National Hematology and Transfusiology Center
2 Azerbaijan National Hematology and Transfusiology Center
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        "resumen" => "<span id="abss0001" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="cesectitle0001">Objective</span><p id="spara001" class="elsevierStyleSimplePara elsevierViewall">Ghosal hematodiaphyseal dysplasia syndrome &#40;GHDD&#41; is a rare authosomal ressesive disorder characterized by increased bone density and regenerative corticosteroid-sensitive anemia&#46;We describe GHDD in an 11-year old Azerbaijani boy with refractory anemia&#44;mild thrombocytopenia and radiological metadiaphyseal dysplasia&#46;The diagnosis was made based on clinical and laboratory examinations and genetic analysis&#46;We have observed a significant improvement of anemia after administration of steroids&#46;</p></span> <span id="abss0002" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="cesectitle0002">Case report</span><p id="spara002" class="elsevierStyleSimplePara elsevierViewall">An 11-year-old boy with long-standing anemia&#44; complained of fatigue&#44;delayed physical development&#44;and limited range of motion in the joint&#46;Physical examination did not reveal LAP and hepatosplenomegaly&#46;Among the dysmorphic craniofacial changes mentioned in the literature&#44; has a tower-shaped skull&#44;micrognotia&#44;drooping ears&#44;a long and wide philtrum&#44;and a thin upper lip&#46;Skeletal X-ray imaging showed fibrotic changes and varying degrees of osteopenia in the metaphysis of the long tubular bones&#46;</p></span> <span id="abss0003" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="cesectitle0003">Methodology</span><p id="spara003" class="elsevierStyleSimplePara elsevierViewall">The blood count&#58; Hb 7&#46;0 g&#47;dl&#44;HCT 24&#46;5&#37;&#44;reticulocytes 5&#46;6&#37;&#44;MCV 78fL&#44;MCHC 28&#46;6 g&#47;dl&#44;WBC count 6860&#47;mm3&#44;platelets 165000&#47;mm3&#44;ESR 75 mm&#47;h&#44;anisocytosis in erythrocytes and platelets were observed in a peripheral blood smear&#46;Hemoglobin electrophoresis&#44;iron studies&#44;vitamin B12 and folic acid were normal&#46;Coombs test was negative&#46;Bone marrow examination showed hypoplasia in erythroid and megakaryocytic series and dysgranulocytopoiesis&#46;</p></span> <span id="abss0004" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="cesectitle0004">Results</span><p id="spara004" class="elsevierStyleSimplePara elsevierViewall">After detection of exon 12 &#40;&#40;p&#46;Gly473Trp&#41;&#44;rs149988492&#44;CM215867&#41; in the genetic panel analysis of anemia&#44;steroid treatment at a dose of 1 mg&#47;kg&#47;day was started and anemia improved at 1-month follow-up &#40;Hb level 6&#46;8 g&#47;dL to 11&#46;9 g&#47;dL&#41;&#44;but mild thrombocytopenia was noted to persist&#46;The clinically insignificant CRP elevation normalized during the treatment&#46;</p></span> <span id="abss0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="cesectitle0005">Conclusion</span><p id="spara005" class="elsevierStyleSimplePara elsevierViewall">GHDD should be considered in patients with clinical and radiographic evidence of diaphyseal dysplasia as well as hematological abnormalities&#46; In addition&#44; bone dysplasia should be investigated in treatment-resistant hematological pathologies of unknown origin&#46; Although GHDD is rare&#44; clinicians should be informed that it responds well to steroid therapy&#46;</p></span>"
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Article information
ISSN: 25311379
Original language: English
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Idiomas
Hematology, Transfusion and Cell Therapy