Granulocytic sarcoma is an uncommon tumor composed of granulocytic precursor cells. Because it occurs in a variety of clinical settings and because the tumor cells are primitive it is frequently unrecognized during life. This presentation details the authors' experience with 61 biopsy-proven granulocytic sarcomas. The patient age range was from 2 to 81 years (mean 48 years). In eight patients the tumors were multiple. Most common sites of involvement were bone, periosteum, soft tissue, lymph node and skin. Twenty-two tumors occurred in 15 patients with no known disease, 26 occurred in 24 patients with a known myeloproliferative disorder, and 13 occurred in 11 patients with proven acute myeloid leukemia. Thirteen of the 15 patients with no known disease developed acute leukemia in from one to 49 months after the biopsy of their tumors (mean 10 months). Most tumors occurring in patients with a known myeloproliferative disorder were associated with blast crisis. The authors' cases displayed a morphologic range from well-differentiated to those tumors that displayed virtually no evidence of differentiation by conventional microscopy. It was therefore not surprising that most tumors were originally diagnosed as lymphoma. Chloro-acetate esterase (CAE) stains were performed on 56 tumors and 47 were studied with antilysozyme immunoperoxidase technique. Fifty-six of the 57 specimens studied by either technique were positive. Antilysozyme immunoperoxidase stains were particularly useful in confirming the diagnosis.

Based on a 6 1/2-year study of 284 consecutive adult patients with primary myelodysplastic syndrome (MDS) and and acute myelogenous leukaemia (AML), we have found that refined chromosome analysis can be used as an independent prognostic indicator in the great majority of patients with MDS and AML. In MDS, the FAB subtype was also found to have prognostic value and this was enhanced when the chromosomal findings were taken into consideration. In AML, the age of the patient correlated more closely with the chromosomal changes in predicting prognosis in most patients than did the FAB classification. Previously we reported that refined chromosome analysis of bone marrow specimens from 161 adult patients with primary or non-therapy related MDS and AML identified three prognostic chromosomal categories in each disease, representing 40% of all patients (Yunis et al, 1984, 1986). By extending our study to 284 patients, as well as a longer follow-up, it was possible to determine the prognostic implications of two additional chromosomal categories in MDS and five in AML. Since 73% of all patients are now represented in well-defined chromosomal subgroups with prognostic significance, refined chromosome analysis emerges as a tool that could have considerable impact in protocols.

Over an 18-year period a distinctive large cell lymphoreticular neoplasm (Richter's transformation) developed in 9 patients with chronic lymphocytic leukemia. Clinical findings at the onset of Richter's transformation were remarkably uniform and consisted of the abrupt onset of fever, marked asymmetric lymphadenopathy with the formation of masses, splenomegaly, and hepatomegaly. All patients underwent rapid clinical deterioration followed by death within six and a half months. Earliest infiltrates of large lymphoreticular cells were identified in the lymph nodes in 3 of 4 patients and the bone marrow in 3 of 9 patients, while no patient had peripheral blood involvement. Autopsy examinations revealed extensive infiltrates of large lymphoreticular cells, predominantly in bone marrow, lymph nodes, liver, spleen, but also in kidney, lung, and gastrointestinal tract. In each case, these large lymphoblast-like and pleomorphic lymphoreticular cells were admixed with mature-appearing lymphocytes and intermediate forms (prolymphocytes). Electron microscopic and immunoperoxidase studies provided additional evidence that this highly aggressive lymphoreticular neoplasm represents a transformation or dedifferentiation of chronic lymphocytic leukemia.