Document Detail

Blood findings in generalized mastocytosis: evidence of frequent simultaneous occurrence of myeloproliferative disorders.
MedLine Citation:
PMID:  2128807     Owner:  NLM     Status:  MEDLINE    
Blood findings in 61 cases of generalized mastocytosis (GM) were evaluated. The cases were divided into two major variants: Systemic mastocytosis (SM; n = 34) with urticaria pigmentosa-like skin lesions, and malignant mastocytosis (MM; n = 27), without skin involvement. The following results were obtained: (1) Significant differences between MM and SM were found in the main haematological parameters (erythrocyte, platelet and leucocyte counts and haemoglobin level); normal values were found in 16 of the SM cases, but never in MM. (2) The main pathological findings were: in SM, anaemia (9/34) and leucocytosis (5/34); and in MM, leucocytosis (19/27), monocytosis (14/27), eosinophilia (12/27), bicytopenia (12/27, mostly anaemia with thrombocytopenia), basophilia (10/27) and isolated anaemia (7/27). (3) The major finding was a significant difference between MM and SM in the incidence of myeloproliferative disorders (MPD), myelodysplasia and mast cell leukaemia (MCL): these disorders occurred in 23 (92%) MM patients, but only in two (6%) SM patients (P less than 0.001). The four instances of MCL and two of myelodysplasia all occurred with MM. Of the 19 cases of MPD, six (SM, 1; MM, 5) were acute variants (acute myeloid and myelomonocytic leukaemias) and 13 (SM, 1; MM, 12) were chronic variants. No case of malignant lymphoma was noted. (4) The blood picture in 10 of 13 chronic MPD cases represented an atypical chronic myeloid leukaemia for which the preliminary descriptive term 'mastocytosis-associated MPD' is proposed. (5) A survey of 103 published cases (SM, 77; MM, 26) yielded similar findings, including a high incidence of MPD and MCL in MM. These findings add further weight to the argument for recognizing SM and MM as two separate entities.
H P Horny; M Ruck; M Wehrmann; E Kaiserling
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Publication Detail:
Type:  Comparative Study; Journal Article    
Journal Detail:
Title:  British journal of haematology     Volume:  76     ISSN:  0007-1048     ISO Abbreviation:  Br. J. Haematol.     Publication Date:  1990 Oct 
Date Detail:
Created Date:  1991-07-02     Completed Date:  1991-07-02     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0372544     Medline TA:  Br J Haematol     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  186-93     Citation Subset:  IM    
Institute of Pathology, Eberhard-Karls University, Tübingen, Federal Republic of Germany.
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MeSH Terms
Erythrocyte Count*
Hematologic Diseases / blood,  complications
Hemoglobins / analysis
Leukemia, Mast-Cell / blood,  complications
Leukocyte Count*
Mastocytosis / blood*,  complications
Middle Aged
Myeloproliferative Disorders / blood*,  complications
Platelet Count*
Reg. No./Substance:
Comment In:
Br J Haematol. 1991 Mar;77(3):436   [PMID:  2012771 ]

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