Document Detail

Splenic pathology in myelodysplasia: a report of 13 cases with clinical correlation.
MedLine Citation:
PMID:  9777988     Owner:  NLM     Status:  MEDLINE    
Splenomegaly is uncommon in myelodysplasia (MDS) and, although cytopenias may be severe, therapeutic splenectomy is rarely performed. We report the histologic, histochemical, and immunophenotypic findings of nine cases of surgical splenectomy and four postmortem spleens from MDS patients. Four histologic patterns were identified: one dominated by erythrophagocytosis, one characterized by red pulp plasmacytosis, one with extramedullary hematopoiesis as the only salient finding, and one with marked red pulp expansion caused by a monocytic proliferation. Wright-Giemsa and histochemical stains were performed on touch preparations in three cases and played a critical role in the precise subclassification of one MDS patient's hematologic disorder, which ultimately proved to be chronic myelomonocytic leukemia. Splenectomy led to sustained improvement of cytopenias in three cases, but did not eliminate transfusion dependence for the remaining patients. Three splenectomy cases exhibited clustered Leder-negative mononuclear elements: two of these patients experienced disease progression to refractory anemia with excess blasts in transformation or acute myelogenous leukemia during post-splenectomy follow-up, whereas none of the three splenectomy patients without clustered mononuclear elements did. We conclude that splenomegaly in MDS usually reflects the sequelae of dyspoiesis rather than evidence of a proliferative phase, that clustering of Leder-negative large cells may correlate with either a substantial monocytic component or, possibly, increased risk of disease progression, and that the spleen can provide diagnostic as well as prognostic information in MDS patients with splenomegaly.
M D Kraus; N L Bartlett; M D Fleming; D M Dorfman
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  The American journal of surgical pathology     Volume:  22     ISSN:  0147-5185     ISO Abbreviation:  Am. J. Surg. Pathol.     Publication Date:  1998 Oct 
Date Detail:
Created Date:  1998-10-28     Completed Date:  1998-10-28     Revised Date:  2005-11-16    
Medline Journal Info:
Nlm Unique ID:  7707904     Medline TA:  Am J Surg Pathol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1255-66     Citation Subset:  IM    
Department of Pathology, Washington University School of Medicine, St. Louis, Missouri 63110, USA.
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MeSH Terms
Aged, 80 and over
Bone Marrow Cells / pathology
Immunoenzyme Techniques
Leukemia, Myelomonocytic, Chronic / metabolism,  pathology*,  surgery
Leukocytes, Mononuclear / pathology
Middle Aged
Myelodysplastic Syndromes / metabolism,  pathology*,  surgery
Peroxidase / metabolism
Retrospective Studies
Spleen / metabolism,  pathology*,  surgery
Splenomegaly / pathology*,  surgery
Reg. No./Substance:

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