Barbara H. Tindle

Immunoblastic Lymphadenopathy a Hyperimmune Entity Resembling Hodgkin's Disease

Immunoblastic lymphadenopathy, although it resembles Hodgkins disease, is a distinct, hyperimmune disorder apparently of the B-cell system. In 32 cases, it was characterized by a morphologic triad: proliferation of arborizing small vessels; prominent immunoblastic proliferations; and amorphous acidophilic interstitial material. Clinically, it is manifested by fever, sweats, weight loss, occasionally a rash, generalized lymphadenopathy and often hepatosplenomegaly. There is a consistent polyclonal hyperglobulinemia and often hemolytic anemia. The course of the disease is usually progressive, with a median survival of 15 months in 18 fatal cases. The cellular proliferation appears benign morphologically in the pretherapy biopsies and in 10 of 12 available autopsy cases. In three cases the process evolved into a lymphoma of immunoblasts, immunoblastic sarcoma. The basic process appears to be a non-neoplastic hyperimmune proliferation of the B-cell system involving an exaggeration of lymphocyte transformation to immunoblasts and plasma cells that may be triggered by a hypersensitivity reaction to therapeutic agents.

Convoluted lymphocytic lymphoma in adults: a clinicopathologic entity.

Twelve adults had a distinct clinicopathologic type of malignant lymphoma that closely resembles the mediastinal lymphomas of childhood. Nine patients presented with mediastinal masses, and seven had symptoms related to intrathoracic compression. Seven patients presented with or developed leukemia, and in four of these patients the central nervous system (CNS) became involved. Structurally, the tumor cells had a distinctive stippled chromatin pattern, in addition to the characteristic nuclear convolutions. Tumor cells from five patients were studied immunologically, and, in each case, the tumor cells formed rosettes with sheep erythrocytes. The response to combination chemotherapy was rapid and dramatic, but usually transient, with relapse in the CNS or previously involved sites. The above data strongly suggest that these cases represent a distinct clinicopathologic entity that should be treated similarly to childhood leukemia and lymphoma, with intensive multiple agent induction, CNS prophylaxis, possibly radiation therapy to initially involved sites, and prolonged maintenance.

Lacunar cells of nodular sclerosing Hodgkin's disease: An ultrastructural and immunohistologic study

Tissues from 22 cases of nodular sclerosing Hodgkins disease were studied by light and electron microscopy in conjunction with immunohistologic and cytochemical staining. The presence of lipid in the cytoplasm of lacunar cells suggested that this was responsible for the distinctive “lacunar” appearance of the cells. Marked morphologic similarities between “blast cells” resulting from mitogen stimulation of lymphocytes in vitro, immunoblasts seen in reactive lymphoid tissues, and mononuclear “Hodgkins” cells in Hodgkins disease suggested that all three cell types may result from lymphocyte transformation. It also seemed apparent that there was a developmental sequence from lymphocyte to transformed lymphocyte to the abnormal mononuclear Hodgkins cell, with further progression, through increasing size and nuclear lobulation, to the lacunar cell or, alternatively, to the diagnostic Reed‐Sternberg cell. This proposed sequence was supported by immunoperoxidase studies in which cytoplasmic immunoglobulin was demonstrated in mononuclear Hodgkins cells, lacunar cells and Reed‐Sternberg cells. The proposed relationship between these cells was also supported by the findings of both kappa and lambda chains in the same cells, a pattern not seen in reactive transformed lymphocytes.

Case 30-1977

Presentation of Case A 60-year-old woman was admitted to the hospital because of fever and lymphadenopathy. She was well until one month earlier, when a mild sore throat developed, with fever, enla...

Childhood lymphoma-leukemia. I. correlation of morphology and immunological studies

Acute lymphocytic leukemia of childhood (ALL) is a heterogeneous disorder. Furthermore, the related lymphomas have been separated arbitrarily according to clinical presentation. This study is based on a combined clinical, morphological (cytological) and immunological evaluation of 49 cases of childhood lymphoma or leukemia. We have identified three separate groups, which in the past have not always been clearly distinguished, but which do appear to have distinctive clinical and cytological features: 1) convoluted lymphocytic lymphoma/leukemia, of probable T cell origin (7 cases), usually associated with thoracic involvement; 2) small noncleaved follicular center cell (FCC) (Burkitt‐like) lymphoma/leukemia, of B cell origin (6 cases), usually associated with abdominal involvement; 3) a heterogeneous (ALL) group (36 cases). This study demonstrates that differentiation of these three entities can often be made on the basis of cytological examination and the features of clinical presentation. It remains to be determined whether these sub‐groups, and other more subtle variations in surface marking (E rosettes) and morphology within the ALL group, reflect important differences in behavior pertinent to selection of therapy.