Extramedullary myeloid cell tumors arising in the setting of chronic myelomonocytic leukemia. A report of two cases.

We report two cases of extramedullary myeloid cell tumor that arose in patients with chronic myelomonocytic leukemia. In both cases, the tumors were difficult to recognize histologically because the neoplasms lacked cytological evidence of granulocyte maturation, such as cytoplasmic granulation or eosinophilic myelocytes, and the Leder stains for chloroacetate esterase were negative. Immunohistochemical studies were necessary to establish the correct diagnosis. The neoplastic cells in both tumors expressed myeloperoxidase, lysozyme, and CD43 and were negative for B-cell, T-cell, and other nonhematopoietic antigens tested. We report these cases to emphasize that extramedullary myeloid cell tumors may rarely precede transformation to acute myeloid leukemia in patients with chronic myelomonocytic leukemia. Extramedullary myeloid cell tumors of monocytic lineage may be difficult to recognize in routine and Leder-stained sections, and immunohistochemical studies may be essential for establishing the diagnosis.

Bone marrow involvement in large-cell lymphoma. Prognostic implications of discordant disease.

Discordant lymphomas are those in which two different histologic subtypes of non-Hodgkin's lymphoma are present simultaneously in the same patient at two or more separate disease sites. Discordance usually involves a lower grade follicular lymphoma in one anatomic site and a higher grade diffuse lesion elsewhere. A common type of discordance is seen in patients with a primary diagnosis of diffuse large-cell lymphoma (DLCL) who demonstrate bone marrow involvement by a lower grade lesion, such as a small cleaved cell or mixed small cleaved and large cell lymphoma. This study was undertaken to assess retrospectively the clinical implications of such bone marrow involvement, as well as the possible biologic mechanisms. Of the 59 DLCL cases studied, 20 (33.9%) showed evidence of bone marrow involvement, 14 of which were discordant (70%). The most significant findings included the following: Overall treatment responses and survivals in discordant patients with predominantly small cleaved cells in the marrow were similar to those in patients with no marrow involvement (mean survivals, 47.7 and 49 months, respectively), and were significantly longer than in patients with concordant marrow involvement (mean survival, 13.1 months, P < .05). Patients with discordant marrow infiltrates composed of a mixed cell population tended to do as poorly as those with concordant involvement. No clear-cut pattern of relapse in discordant patients was found, but persistence of small cleaved cells in some was reminiscent of lower grade B-cell lesions. Other features associated with lower grade lesions included older age, less incidence of central nervous system involvement, and lesser extent and proportion of marrow infiltration. Finally, in approximately half the cases with discordant involvement, lymphoma was present unilaterally, emphasizing the need to perform bilateral biopsies for staging.

Renal leiomyosarcoma: plea for aggressive therapy.

We report a case of renal leiomyosarcoma arising from the renal pelvis with immunohistochemical confirmation of the diagnosis. Treatment was extirpative surgery for both primary and metastatic lung lesions as well as adjunctive radiation and chemotherapy. This patient remains disease-free twelve months following treatment.

Treatment of malignant Leydig cell tumor.

Malignant Leydig cell tumors (LCT) are rare. Only 32 cases of malignant LCT (as evidenced by metastatic spread) were reported. Generally metastatic spread occurs within 2 years of the primary LCT, and the patient dies within 2 years of the discovery of metastatic disease. The tumor is highly resistant to both radiation and chemotherapy. It also has a great propensity for recurring after surgical resection. A case is reported of a patient whose metastatic disease occurred 8 years after his primary LCT had been resected. He was treated with doxorubicin and mitotane without response. The clinical features of this case are highlighted, and a review of the literature describing treatment of this rare disease is presented.

Mucopolysaccharidosis I presenting with endocardial fibroelastosis of infancy.

We describe two female infants with Hurler syndrome (mucopolysaccharidosis I) whose deaths are attributed to cardiac failure with associated, autopsy-confirmed endocardial fibroelastosis. One infant had confirmed alpha-L-iduronidase deficiency in cultured dermal fibroblasts, and the other infant had histologic evidence of tissue mucopolysaccharide accumulation at autopsy and a sibling with confirmed alpha-L-iduronidase deficiency and the Hurler syndrome phenotype. Clear cells ("Hurler" cells) were identified within the myocardium and endocardium of both infants. We propose that the ventricular mural accumulation of mucopolysaccharides induced extensive proliferation of elastic or collagen fibers within the endocardium. Cardiac failure may precede recognition of clinical and roentgenographic features of Hurler syndrome. Our findings and a literature review suggest that certain heritable storage disorders, including mucopolysaccharidosis I, should be considered when infants have clinical electrocardiographic and echocardiographic findings consistent with endocardial fibroelastosis or have autopsy-documented endocardial fibroelastosis.

Induction of immunologic memory by a lipopolysaccharide-protein complex isolated from Fusobacterium necrophorum: humoral response.

The serum antibody response in BALB/c mice to a lipopolysaccharide-protein (LPS-P) complex was monitored by the enzyme-linked immunosorbent assay, total and 2-mercaptoethanol-resistant hemagglutination, and radial immunodiffusion. Dose-response analyses demonstrated that suitable primary doses of LPS-P injected IV or IM induced substantial concentrautions of specific serum immunoglobulin (Ig) M and IgG. Moreover, these values were greatly enhanced with small-dose booster injections. Inoculation of mice with a suitable primary IM dose of aluminum hydroxide-precipitated LPS-P-induced specific IgM and IgG amounts that were detectable for 120 days. An enhanced secondary response to antigen booster injections was generated 105 days after primary inoculation, providing direct evidence that LPS-P can induce immunologic memory. Similar results were obtained for IV inoculations of LPS-P, although the primary IgG response was not as persistent. Seemingly, the memory response to LPS-P was largely dependent on the protein component of the molecule.

Induction of immunologic memory by a lipopolysaccharide-protein complex isolated from Fusobacterium necrophorum: cellular response.

The ability of a lipopolysaccharide protein (LPS-P) complex extracted from Fusobacterium necrophorum to establish immunologic memory in BALB/c mice splenocytes was demonstrated. The LPS-P molecule differed from the phenol water-extracted LPS because it contained approximately 12% protein. Initial experiments showed that primary and secondary spleen plaque-forming cell (PFC) responses to IV or IM injections of LPS-P were highly dose-dependent. Suitable primary doses stimulated significant (P less than 0.05) amounts of direct and direct + indirect PFC by postinoculation day (PID) 14 and primed the mice for an enhanced secondary response to small booster injections. When mice were inoculated with a suitable primary IM dose of aluminum hydroxide-precipitated LPS-P, significant amounts of direct and direct + indirect PFC were detectable through PID 120. Moreover, significant enhancement of these values was attained with an IV booster injection at PID 105. Primary IV inoculation with LPS-P produced similar results, although the primary response was not as persistent.