Acquired idiopathic pure white cell aplasia: A rare cause of agranulocytosis
Pure white cell aplasia (PWCA) is an exceedingly rare hematological disease of unknown origin exemplified by agranulocytosis, virtually lack of all granulocytic spectrums (from myeloblast to neutrophils) in the bone marrow with normal erythroid and megakaryocytic lineages. It has been associated wit...
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Wolters Kluwer Medknow Publications
2014-01-01
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doaj-997015318a3f4225b58f3a69cc5275b22020-11-24T20:45:15ZengWolters Kluwer Medknow PublicationsJournal of Applied Hematology1658-51272014-01-015416116310.4103/1658-5127.146952Acquired idiopathic pure white cell aplasia: A rare cause of agranulocytosisSadia SultanSyed Mohammad IrfanPure white cell aplasia (PWCA) is an exceedingly rare hematological disease of unknown origin exemplified by agranulocytosis, virtually lack of all granulocytic spectrums (from myeloblast to neutrophils) in the bone marrow with normal erythroid and megakaryocytic lineages. It has been associated with underlying autoimmune disorders, thymomas, chronic lymphocytic leukemia and drug induced or may be idiopathic. Here we report the case of a patient with this rare finding, who was admitted to our hospital with septicemia having agranulocytosis, multiple oral candidiasis, perianal abscess and right arm cellulitis. Bone marrow examination revealed absolute absence of all myeloid precursors cells. All diagnostic workup for secondary underlying cause were found to be negative and diagnosed as idiopathic PWCA. Immunosuppression with methylprednisolone pulse therapy was failed to induce neutrophils recovery. PWCA is a benign disorder, but have serious consequences due to threatening febrile neutropenia, disseminated fungal infection and septicemia. Needs urgent management depending upon underlying etiology.http://www.jahjournal.org/article.asp?issn=1658-5127;year=2014;volume=5;issue=4;spage=161;epage=163;aulast=SultanAgranulocytosisidiopathicpure white cell aplasia |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Sadia Sultan Syed Mohammad Irfan |
spellingShingle |
Sadia Sultan Syed Mohammad Irfan Acquired idiopathic pure white cell aplasia: A rare cause of agranulocytosis Journal of Applied Hematology Agranulocytosis idiopathic pure white cell aplasia |
author_facet |
Sadia Sultan Syed Mohammad Irfan |
author_sort |
Sadia Sultan |
title |
Acquired idiopathic pure white cell aplasia: A rare cause of agranulocytosis |
title_short |
Acquired idiopathic pure white cell aplasia: A rare cause of agranulocytosis |
title_full |
Acquired idiopathic pure white cell aplasia: A rare cause of agranulocytosis |
title_fullStr |
Acquired idiopathic pure white cell aplasia: A rare cause of agranulocytosis |
title_full_unstemmed |
Acquired idiopathic pure white cell aplasia: A rare cause of agranulocytosis |
title_sort |
acquired idiopathic pure white cell aplasia: a rare cause of agranulocytosis |
publisher |
Wolters Kluwer Medknow Publications |
series |
Journal of Applied Hematology |
issn |
1658-5127 |
publishDate |
2014-01-01 |
description |
Pure white cell aplasia (PWCA) is an exceedingly rare hematological disease of unknown origin exemplified by agranulocytosis, virtually lack of all granulocytic spectrums (from myeloblast to neutrophils) in the bone marrow with normal erythroid and megakaryocytic lineages. It has been associated with underlying autoimmune disorders, thymomas, chronic lymphocytic leukemia and drug induced or may be idiopathic. Here we report the case of a patient with this rare finding, who was admitted to our hospital with septicemia having agranulocytosis, multiple oral candidiasis, perianal abscess and right arm cellulitis. Bone marrow examination revealed absolute absence of all myeloid precursors cells. All diagnostic workup for secondary underlying cause were found to be negative and diagnosed as idiopathic PWCA. Immunosuppression with methylprednisolone pulse therapy was failed to induce neutrophils recovery. PWCA is a benign disorder, but have serious consequences due to threatening febrile neutropenia, disseminated fungal infection and septicemia. Needs urgent management depending upon underlying etiology. |
topic |
Agranulocytosis idiopathic pure white cell aplasia |
url |
http://www.jahjournal.org/article.asp?issn=1658-5127;year=2014;volume=5;issue=4;spage=161;epage=163;aulast=Sultan |
work_keys_str_mv |
AT sadiasultan acquiredidiopathicpurewhitecellaplasiaararecauseofagranulocytosis AT syedmohammadirfan acquiredidiopathicpurewhitecellaplasiaararecauseofagranulocytosis |
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