Lymphomas of the mucosa-associated lymphoid tissue. Signet ring cell lymphomas presenting in mucosal lymphoid organs.

Four cases of lymphoma are reported which were postulate to have developed from neoplastic transformation of the lymphoid cells of the mucosa-associated lymphoid tissue (MALT). This contention is primarily based on two observations: the peculiar "homing" tendency of these lymphomas and the immunoglobulin isotype. Both properties are characteristic of the lymphoid cells of the MALT. These lymphomas can also be identified by their histologic structure (signet ring cell lymphomas) and their presentation in an MALT organ. These lymphomas are probably separable from other lymphomas proposed to arise from lymphocytes of the gut-associated lymphoid system (GALT).

Randomized study for the treatment of adult advanced Hodgkin's disease: mechlorethamine, vincristine, procarbazine, and prednisone (MOPP) versus lomustine, vinblastine, and prednisone.

Forty-seven patients with advanced Hodgkin's disease were entered in a prospective, randomized trial comparing MOPP (mechlorethamine, vincristine, procarbazine, and prednisone) with a regimen containing lomustine (CCNU), vinblastine, and prednisone (CCNU-VP). Both groups were comparable for the variables of age, stage, substage (symptoms), histology, prior radiation, and sites of involvement. Seventy-two percent of CCNU-VP-treated patients achieved a pathologically documented complete remission (CR) compared to 41% of the MOPP-treated group. Two additional patients treated with MOPP had remission documented only clinically but have been long-term, disease-free survivors. There was a greater frequency of CR in the patients who had received previous irradiation when compared to patients with no prior irradiation. After a median follow-up of greater than 89 months, there is no statistical difference between the two treatment groups in survival (45% for MOPP and 60% for CCNU-VP). Further, no statistical difference in survival for the two treatment groups was noted when compared by histology, stage, or symptoms. The CCNU-VP combination was better tolerated with significantly less nausea and emesis. The alternative drug regimen of CCNU-VP appears to be as effective as MOPP in producing CR and long-term survival in patients with advanced Hodgkin's disease.

The clinical significance of the morphological subdivision of diffuse "histiocytic" lymphoma: a study of 162 patients treated by the Southwest Oncology Group.

We grouped 162 patients wtih advanced, diffuse histiocytic lymphoma (DHL) into various morphological subtypes to ascertain whether there were any significant differences in survival among them. These patients were staged and treated from 1972 to 1977 according to the protocols of the Southwest Oncology Group. Of the 159 patients on whom a consensus on the diagnosis was reached, 115 were classified morphologically as having large non-cleaved, 26 as B-immunoblastic, 9 as large cleaved, and 6 as T-immunoblastic. The 3 remaining patients did not fit any of these subtypes, but each had a single prominent nucleolus in most tumor cells ("prominent nucleolus" type). Morphological subdivision of DHL did not identify any subgroup of patients with a significantly longer survival, but clinical parameters such as stage, symptoms, and type of treatment significantly influenced survival times.

Infections of hairy cell leukemia. Clinical evidence of a pronounced defect in cell-mediated immunity.

We compared infection rates in 12 patients with hairy cell leukemia (a malignant neoplasm for which the cell of origin remains controversial) with rates in 15 patients with chronic lymphocytic leukemia (a known B-lymphocyte neoplasm) recently treated at four Dallas hospitals. We found a significantly higher over-all rate of infections in the patients with hairy cell leukemia (P = 0.004 BY Gehan's variation on the generalized Wilcoxon test). This increased rate was primarily due to a significantly higher rate of infections normally controlled by the cell-mediated immune system (P = 0.005). Despite these findings, five of six patients with hairy cell leukemia who were skin-tested exhibited intact delayed type hypersensitivity, and each of the three patients examined serologically produced antibodies normally in response to recent infections. A review of the case records of 173 previously described patients with hairy cell leukemia, demonstrated a similar predilection of patients with this disease for infections normally controlled by cell-mediated immunity. In this regard, they were similar to previously described patients with Hodgkin's disease. Both over-all infection rates and rates of fatal infection were highest in patients with hairy cell leukemia who received chemotherapy as their sole form of treatment and lowest in those who underwent splenectomy as their only form of antitumor therapy.

Acute lymphocytic leukemia manifesting as splenic rupture.

Pathologic rupture of the spleen is a rare phenomenon in acute lymphocytic leukemia, and its mechanism is unknown. This complication has been seen almost exclusively in men, and at ages much older than those of the majority of patients who have this leukemia; these unusual features are also unexplained. A case of acute lymphoctyic leukemia with pathologic rupture of the spleen in which the pathologic findings suggest that the splenic rupture was due to infiltration and destruction of the splenic capsule by leukemic cells is reported.

Lupus erythematosus in a patient with amyloidosis, adrenal insufficiency, and subsequent immunoblastic sarcoma: demonstration of the LE phenomenon in the lung.

A patient with systemic lupus erythematosus of long duration developed secondary amyloidosis and finally died after the additional complication of malignant lymphoproliferative disease. Multiple system involvement, typical serologic findings, and postmortem evidence substantiated the diagnosis of lupus erythematosus. Amyloid deposition was found in several organs, but was notably extensive in the adrenal cortex. This extensive deposition resulted in adrenal insufficiency, which was diagnosed clinically and treated until the patient's death from lymphoma. The particular nature of the malignant lymphoma is emphasized; a distinctive feature was the disappearance of positive lupus erythematosus cells from the buffy coat and the reduction in titers of relevant serologic tests toward the end of the illness. In spite of this reduction, many hematoxylin bodies and abundant complete lupus erythematosus cells were found in the lungs on postmortem examination.