Original ArticleNonhepatosplenic Extramedullary Hematopoiesis: Associated Diseases, Pathology, Clinical Course, and Treatment
Section snippets
PATIENTS AND METHODS
After approval of this study by the Mayo Foundation Institutional Review Board, we comprehensively searched the institutional database of medical diagnoses and procedures to identify patients diagnosed with any form of EMH from 1975 to 2002. A retrospective chart review was conducted on all patients identified, focusing on those with NHS-EMH diagnosed antemortem by tissue biopsy, FNA biopsy, or radionuclide bone marrow scanning. Patients diagnosed with NHS-EMH by computed tomography or MRI
Associated Diseases
We identified 510 patients as having been diagnosed with EMH. Twenty-seven patients (5.3%) were diagnosed with NHS-EMH and met inclusion criteria: 18 (67%) had MMM (Table 1) and 9 (33%) had no evidence of MMM (non-MMM) (Table 2). The MMM group consisted of 12 patients (67%) diagnosed with agnogenic myeloid metaplasia (AMM) and 6 (33%) diagnosed with post–polycythemic myeloid metaplasia (PPMM) having progressed from polycythemia vera. The non-MMM group consisted of 3 patients with congenital
DISCUSSION
The occurrence of NHS-EMH is rare, and to our knowledge, this study is the largest description of patients with NHS-EMH diagnosed antemortem. Most cases of NHS-EMH occurred in the setting of hematologic disease, with MMM being the most frequent diagnosis. Only 2 patients had no evidence of hematologic disease. Most patients with NHS-EMH had chronic anemia, with a median hemoglobin value of 9.5 g/dL at the time of NHS-EMH diagnosis. This supports the theory that EMH might be a compensatory
CONCLUSIONS
Nonhematosplenic EMH can arise in a variety of organs, producing various symptoms that are sometimes life-threatening. Although rare, NHS-EMH should be suspected in patients with predisposing conditions such as MMM and PPMM. The etiology of EMH remains to be elucidated. Current theories for the development of EMH fail to account for all cases of EMH, especially those arising outside of the reticuloendothelial system. Therefore, we have proposed that a secreted factor, either aberrantly produced
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This work was supported by an American Society of Hematology medical student award.