Search

The spectrum of CD30+ cutaneous lymphoproliferative disorders is characterized by the histology of a high-grade lymphoma but frequent clinical regression of skin lesions in lymphomatoid papulosis (LyP) and occasional regression in CD30+ large cell lymphomas (LCLs). A recent study shows that apoptosis may be a significant mechanism of regression of LyP (Arch Dermatol 133:828-833, 1997). Therefore, we studied expression of proteins that induce apoptosis, including CD27, CD40, CD95, and nerve growth factor receptor (NGF-R), as well as anti-apoptotic protein bcl-2 in skin lesions from 25 patients within the spectrum of CD30+ cutaneous lymphoma. Our results show consistent expression of CD95 (APO-1/Fas), but rare or absent expression of CD27, CD40, and NGF-R on tumor cells from both regressing LyP lesions and nonregressing CD30+ lymphomas. Bcl-2 was expressed at low levels in LyP and at high levels in pleomorphic CD30+ lymphomas. These results indicate that, in addition to CD30, CD95 expression is preferentially expressed at high levels in all cutaneous CD30+ lymphomas and suggest that CD95 may play a role in the regression of CD30+ skin lesions. Expression of bcl-2 appears to protect tumor cells from apoptosis in CD30+ lymphoproliferative disorders.

We report the case of a 42-year-old man who presented with B-symptoms, moderate splenomegaly and multiple nodules in the liver. Histologically, lymphocytic infiltrates were seen in the portal spaces and sinusoids of the liver and in the paratrabecular areas of the bone marrow. After excision, the spleen showed minimal disturbance of architecture with an expansion of the follicular marginal zones. These findings were considered inconclusive for lymphoma and the patient was treated only with non-steroidal anti-inflammatory drugs for persisting fever. Five months after splenectomy, a bone marrow biopsy still showed diffuse lymphoid infiltrates. From then on, the patient's condition improved with no further evidence of disease. Ten years after splenectomy the case was reconsidered as a splenic marginal cell lymphoma, indolent variant. Immunohistochemical and gene rearrangement studies demonstrated the monoclonality of the splenic proliferation, supporting the diagnosis. A further bone marrow biopsy did not detect residual lymphoid infiltrates. This case confirms that splenic marginal zone cell lymphoma may have a deceptively favorable course, even when presenting at an advanced stage. Moreover, it indicates that extrasplenic localizations of the lymphoma may persist for a long while after splenectomy but may vanish over time without therapy.

Splenic marginal zone (MRZ) cell lymphoma is a recently described neoplasm arising in a unique compartment of splenic white pulp, producing massive splenomegaly and spreading to bone marrow and distant lymph nodes. We report three cases of splenic lymphoma that morphologically and immunohistochemically appear to originate from MRZ cells that presented as indolent neoplasms involving the spleen but with no or only moderate enlargement of the organ, presumably representing an early clinical stage of this disorder. Despite the evidence of involvement of the liver in one case, lymph nodes and bone marrow proved to be uninvolved. Histologically, the three spleens showed similar features, being characterized by the involvement of white pulp follicles and periarteriolar lymphoid sheaths by medium-sized lymphoid cells with slightly irregular nuclei and ample cytoplasm. Immunohistochemically, all the specimens expressed a series of B-lineage markers that, in contrast to specimens of monocytoid B cell lymphoma (MBCL) and hairy cell leukemia (HCL) studied for comparison, did not react with KiB3, LN1, and DBA.44 monoclonal antibodies.

In this report we analyze the morphological and immunohistochemical findings observed in 5 cases of CD30/Ki-1 positive anaplastic large cell lymphoma, a recently recognized neoplastic entity. In comparison with the Ki-1 lymphomas so far described, these cases showed a fairly large number of Reed-Sternberg-like cells, often admixed with small lymphocytes and occasional eosinophils. Moreover, in all our cases immunohistochemical reactions detected the CD15/Leu-M1 antigen, together with markers of the T-lineage and of lymphoid activation. In previous studies the CD15/Leu-M1 antigen has been found in the majority of cases of Hodgkin's disease, but has been stated to be absent typically in Ki-1 lymphomas. Our results indicate that this antigen cannot be considered a reliable tool to distinguish between Ki-1 lymphomas and Hodgkin's disease. Furthermore, the morphological and immunohistochemical findings reported suggest that in some cases Ki-1 cell lymphoma and Hodgkin's disease may be closely related. They may represent different steps in the progression of the same lymphoproliferative disorder.

Thirty adult patients with lymphoblastic lymphoma were treated with two different programs according to bone marrow findings. Bone marrow positive patients were given an ALL-like program: vincristine, daunorubicin, cyclophosphamide and prednisone for induction of remission, CNS prophylaxis, continuous maintenance for three years and monthly reinductions. Bone marrow negative patients were given a conventional lymphoma program with CHOP-Bleo and limited RT on bulky mediastinum without CNS prophylaxis. The CR rate of the whole group was 54% (62% for ALL-treated versus 47% for lymphoma-treated patients; not significantly different), with a median survival for remitters of 28.5 mos. Relapse-free survival of the whole group was 65% at 12 and 25% at 24 mos. Stage IV ALL-treated patients had a median survival of 16.5 versus 10 mos for stage IV lymphoma-treated ones (p = 0.05); the three-years survival was 24 and 10%, respectively. No patients undergoing CNS prophylaxis (ALL-therapy) had neurological complications or late meningeal relapse. The better prognosis of ALL-treated patients, in spite of bone marrow positivity, argues in favor of an ALL-like therapy in all adult lymphoblastic lymphomas, in terms of CR rate, overall survival, and absence of CNS relapse. This therapy must be adopted irrespective of bone marrow findings, and regardless of how localized the lymphoma appears to be.

We reviewed 182 consecutive adult patients with low-grade malignancy, non-Hodgkin's lymphomas classified according to the Kiel classification, followed at the Division of Hematology, Policlinico S. Matteo, Pavia, from January 1975 to December 1981, to recognize, in each histopathologic type, important subgroups from the prognostic standpoint. Median follow-up was 36 months. No significant differences were observed in the response rate to conventional therapy (radiotherapy for localized disease, CVP for advanced stages) between the 4 cytologic types. The centrocytic-centroblastic lymphoma with diffuse nodal architecture showed an intermediate-grade malignancy (median survival, 50 months) and underwent cytologic progression to the high-grade malignancy centroblastic type in 10% of the cases. Large-cell centrocytic and polymorphic lymphoplasmacytoid lymphomas had a poor prognosis (median survival less than 30 months) when treated with conventional therapy for favorable histologies, and 6% of the cases transformed into the high-malignancy immunoblastic type. Patients with lymphocytic lymphoma with bulky mediastinum had a median survival of 20 months. The identification of these subgroups with a worse prognosis may have therapeutic implications.