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Chediak-Higashi syndrome

Chédiak-Higashi syndrome is a rare childhood autosomal recessive disorder that affects multiple systems of the body, which arises from a mutation that causes silvery hair. It is a disease with impaired leukocyte function due to failure of phagolysosome formation – As a result of disordered intracellular trafficking there is impaired lysosome degranulation with phagosomes. In addition secretion of lytic secretory granules by cytotoxic T cells is also affected. The disease is characterised by large lysosome vesicles in phagocytes who have poor bactericidal function leading susceptibility to infections, abnormalities in nuclear structure of leukocytes, anaemia, hepatomegaly.

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Clinical hematology atlas
From Archives of Pathology & Laboratory Medicine, 11/1/99 by Pooley, Robert J Jr

By Jacqueline H. Carr and Bernadette F Kodak, 240 pp, with 356 illus, $39, Philadelphia, Pa, WB Saunders Co, 1999.

The goal of the authors, as stated in the preface, is to provide an exceptional reference work for the morphology of normal hematopoiesis and marrow elements, as well as selected benign and malignant morphologic alterations. The book is organized well, simple in design, and is effective as an aid for the inexperienced reviewer and for daily use.

The atlas begins with a chapter addressing the preparation and examination of peripheral blood smears. The text does not attempt to outline all aspects of staining and slide preparation, but it does provide enough information to enable the reader to evaluate smear and staining quality adequately. A short section on the Wright-Giemsa staining procedure is also included.

Subsequent chapters outline the development of each cell line in the bone marrow and are the strong point of the text. Artistic representations of each stage of development are accompanied by corresponding photomicrographs, electron micrographs, numerical values, written descriptions, and reference ranges for bone marrow differentials. The photomicrographs are in color and are of excellent quality. The electron micrographs are also excellent, although their inclusion adds more to the understanding of morphologic features than to practical use. These chapters would be very helpful for teaching or self-study at the microscope.

The last several chapters are devoted to benign and malignant morphologic alterations that may be seen in the peripheral blood and bone marrow. The chapters dealing with erythrocyte variations are good, particularly the section on red cell inclusions. The chapter on diseases affecting erythrocytes, as stated by the authors, is not intended to be a complete reference for conditions such as the thalassemias and Bares hemoglobin. Even so, it could be rendered more practical in future editions by providing multiple representative photomicrographs of each disease or by highlighting only key features that should alert the investigator to the possible presence of one of the various conditions. The chapters discussing changes in leukocytes are very good and provide examples of conditions such as May-Hegglin anomaly and Chediak-Higashi syndrome. Differential features are offered for distinguishing between monocytes and reactive lymphocytes. The acute leukemias, myeloid and lymphoid, are presented in separate chapters, which include criteria for FrenchAmerican-British classification. Two chapters are devoted to the myeloproliferative and the myelodysplastic disorders. These 2 groups of disorders are well outlined for the student or practitioner of hematology and provide a good framework for future learning. Features for classification of the myelodysplastic disorders are presented in chart form and are followed by several high-quality photomicrographs demonstrating the primary morphologic alterations.

The final chapters address the malignant lymphoproliferative disorders, microorganisms, cytochemical stains, and miscellaneous cells that may be seen in the peripheral blood and bone marrow. Classic examples are provided for the lymphoproliferative disorders. The other chapters are brief, but are adequate for an introductory text.

Overall, Clinical Hematology Atlas is a sound text for students, residents, and technologists and should be a helpful reference for routine laboratory use.

Copyright College of American Pathologists Nov 1999
Provided by ProQuest Information and Learning Company. All rights Reserved

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