Pseudo Chediak-Higashi Anomaly

Apply

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

Sarkoidozda Kemik ve Kemik İliği Tutulumu

Dose Adjustment Helps Obtain Better Outcomes in Multiple Myeloma Patients with Bortezomib, Melphalan, and Prednisolone (VMP) Treatment

Objective: Multiple myeloma (MM) has a better survival outcome because of the development of drugs. However, equivalent outcomes cannot be expected from the same drug. Therefore, how the treatment schedule is managed is...

EP ID EP127191
DOI 10.4274/Tjh.2011.0010
Views 99
Downloads 0