Pseudo Chediak-Higashi Anomaly
Journal Title: Turkish Journal of Hematology - Year 2013, Vol 30, Issue 2
Abstract
A 9-year-old girl was admitted to hospital with a 1-month history of fever, weight loss, epistaxis, and abdominal pain.The girl’s parents were non-consanguineous. Her medical history was unremarkable. Upon admission she weighed 27 kg (25th-50th percentile), was 131 cm tall (50th percentile),and was pale. Physical examination showed multiple cervical and inguinal microlymphadenopathies, without hepatosplenomegaly. Complete blood count fi ndings were as follows: hemoglobin 7.9 g/dL; white blood cell count:12.3x109/L; platelet count: 9.2×109/L. Her peripheral blood smear showed 43% blast cells, 42% lymphocytes, 8% monocytes, 6% neutrophils, and 1% eosinophils. Bone marrow aspiration showed hypercellularity, with 45% myeloblasts,15% promyelocytes, 7% myelocytes, 4% metamyelocytes,1% eosinophils, 2% neutrophils, 15% lymphocytes, and 11% normoblasts. Wright staining of a bone marrow smear showed large (2-4 μm) round-to-oval, hyaline-structured eosinophilic granules (pseudo Chediak-Higashi [PCH] granules) within 15% of the blast cells, promyelocytes, and myelocytes (Figure). Some of the granules appeared to be in vacuoles, giving them a haloed appearance. The blasts were myeloperoxidase (MPO)-positive, and periodic acidSchiff (PAS)-negative. Immunophenotypic analysis of the leukemic cells showed proliferation of CD13 (75%), CD33 (59%), CD34 (95%), CD117 (68%), and HLA DR (44%).
Authors and Affiliations
Zekai Avcı, Barış Malbora, Namık Özbek
Keywords
Related Articles
- EP ID EP127191
- DOI 10.4274/Tjh.2011.0010
- Views 90
- Downloads 0
Both Granulocytic and Non-Granulocytic Blood Cells Are Affected in Patients with Severe Congenital Neutropenia and Their Non- Neutropenic Family Members: An Evaluation of Morphology, Function, and Cell Death
Objective: To examine granulocytic and non-granulocytic cells in children with severe congenital neutropenia (SCN) and their nonneutropenic parents. Materials and Methods: Fifteen patients with SCN and 21 nonneutropenic...
Prognostic Factors and a New Prognostic Index Model for Children and Adolescents with Hodgkin’s Lymphoma Who Underwent Autologous Hematopoietic Stem Cell Transplantation: A Multicenter Study of the Turkish Pediatric Bone Marrow Transplantation Study Group
Objective: The prognostic factors and a new childhood prognostic index after autologous hematopoietic stem cell transplantation (AHSCT) in patients with relapsed/refractory Hodgkin’s lymphoma (HL) were evaluated. Materia...
Intradiploic Hematoma in a Hemophilic Patient: Hemophilic Pseudotumor of Calvarium
To the Editor,
Kallikrein İnhibitörü ile Tedavi Edilen Otoeritrosit Sensitizasyon Sendromu
Hb D-Los Angeles [beta121(GH4)Glu>Gln] and Hb Beograd [beta121(GH4)Glu>Val]: Implications for their laboratory diagnosis and genetic origins
Objective: The aim of this study was to determine the laboratory diagnosis and genetic origins of the hemoglobin (Hb) variants, Hb D-Los Angeles and Hb Beograd observed frequently in our region. Material and Methods: Hb...