PubMed · 16079866
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Bruce Diamond. 2005. Profile. Aaron Marcus.. https://doi.org/10.1038/nm0805-816
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Extramedullary myeloid tumors (myeloid sarcomas) are rare neoplasms that are composed of myeloid precursors. They usually arise concurrently with a diagnosis of acute myeloid leukemia, chronic myeloid leukemia, or other myeloproliferative disorders. They may also indicate relapsing disease in a patient with a prior history of leukemia or myeloproliferative disorder. We present our findings of a 63-year-old female diagnosed with extramedullary myeloid tumor first presenting in the gallbladder. She subsequently developed respiratory failure; pre- and postmortem bone marrow studies were negative for leukemia by morphology, flow cytometry, and karyotypic analysis. However, the myeloid neoplasm was disseminated throughout most of her remaining organs. Immunohistochemical stains of the cells indicated a neoplasm of myelomonocytic derivation (CD4, CD43, CD45, CD68, myeloperoxidase, and lysozyme positive). To our knowledge, this is the first report of an extramedullary myeloid neoplasm of the gallbladder with disseminated disease without involvement of the bone marrow.
We report a case of primary leptomenigeal lymphoma (PLML) in an 11-year-old boy presenting with headache, vomiting, and diplopia. The patient was treated on an advanced non-Hodgkin lymphoma protocol with systemic/intrathecal chemotherapy without cranial radiotherapy. He remains in complete remission 33 months after treatment.
BACKGROUND: The TEL-AML1 fusion in precursor-B ALL is generated by a cryptic 12;21 translocation that is detectable by fluorescence in situ hybridization (FISH). It is generally considered a favorable prognostic indicator. Some TEL-AML1+ ALL patients present at diagnosis with extra copies of the fusion, enumerated by FISH. The aim of the study was to determine whether additional copies of TEL-AML1 have clinical significance. PROCEDURE: Charts of all TEL-AML1+ ALL patients at the UM and Children's Hospitals and Clinics of Minnesota between 1996 and 2004 were reviewed. RESULTS: Eight patients (7 males/1 female, mean age 46 months) with two or more TEL-AML1 fusion signals and 24 with single TEL-AML1 fusion signals (18 males/6 females, mean age 52 months) were identified. There was no statistically significant difference in age or gender between the two groups. Patients with double TEL-AML1+ had a higher frequency of myeloid markers CD13 (P = 0.04) or CD33 (P = 0.003) than single TEL-AML1+ patients. Single TEL-AML1+ patients had higher WBC (P = 0.04) than double TEL-AML1+ patients. A trend toward slower therapy response was seen in double TEL-AML1+ patients versus single, (1 of 7 [14%] <5% marrow blasts on Day 7 vs. 13 of 23 [56%], P = 0.09). Double TEL-AML1+ patients had a higher relapse rate (P = 0.09) than single TEL-AML1+ patients. CONCLUSIONS: Utilizing FISH to distinguish subgroups of TEL-AML1 fusion patients may have important prognostic implications. The presence of an extra fusion may portend poorer prognosis. A larger and longer-term follow-up study will be required to verify the possible clinical significance of the presence of multiple TEL-AML1 fusions.