Robert M. Bearman

Malignant lymphoma with primary manifestation in the gonad a clinicopathologic study of 38 patients

This study is based on 38 patients, each of whom had a malignant lymphoma in which the gonad was the site of the main tumor mass at the time of diagnosis. Histiocytic lymphoma was the predominant type in the 27 male patients; in the 11 female patients, poorly differentiated lymphocytic lymphoma was the most frequent type (36%). All of the neoplasms in the males were diffuse, whereas two of the females had neoplasms that were nodular and diffuse. A striking feature was the high frequency of vascular invasion (41%) in the testicular lymphomas, which was reflected in a high incidence (86%) of noncontiguous lung involvement at autopsy, suggesting hematogenous spread. Clinically occult disease is probably responsible for the short interval between the discovery of a gonadal mass and the appearance of generalized disease. A poor prognosis may be expected if there is evidence of generalized disease within one year after diagnosis. Only 2 patients had disseminated disease after a year, whereas all those who died of disseminated disease manifested it within six months after diagnosis. Disease‐free survival times in excess of 60 months in 3 patients who were treated only by orchiectomy or oophorectomy indicate that the gonads may be the primary site of a malignant lymphoma.

Acute (“malignant”) myelosclerosis

This study is based upon an analysis of the hematologic and pathologic material from seven patients with acute myelosclerosis, as well as a review of the literature of 49 cases reported under this designation, or one of its synonyms. Patients with this disease characteristically present with pancytopenia, minimal or absent anisocytosis and poikilocytosis, and a fibrotic bone marrow showing hyperplasia and immaturity of all three cell lines, with particular prominence of megakaryocytes and their precursors. In addition, clinical splenomegaly is almost always absent, and the disease has a rapidly fatal course. We consider only one‐fourth of the cases reported in the literature to have the clinical and hematologic features consistent with the diagnosis of acute myelosclerosis; the remainder represent a variety of myeloproliferative disorders, including chronic myelosclerosis with an accelerated terminal phase, acute myeloblastic leukemia with bone marrow fibrosis, myeloproliferative diseases that cannot be subclassified, and cases in which the data are insufficient for analysis. Using strict clinical and hematological criteria, acute myelosclerosis can be separated from other myeloproliferative disorders as a distinct clinicopathologic entity.

Prolymphocytic leukemia. Clinical, histopathological, and cytochemical observations

The clinical, histopathological, and cytochemical features of eight patients with prolymphocytic leukemia, a rare variant of chronic lymphocytic leukemia, were reviewed. Six of the patients had clinical evidence of “massive” splenomegaly at the time of diagnosis, and in four of these this clinical impression was confirmed by splenic weights in excess of 2000 g. No patient had significant lymph node enlargement. The initial leukocyte count was elevated in seven patients and was greater than 100 × 109/1 in four of them. The absolute prolymphocyte count ranged from 16.3 to 378.1 × 109/1 and was greater than 100 × 109/1 in four patients. Splenectomy in four patients had no lasting effect on the peripheral leukocyte count. In the four patients in whom the disease was shown by surface marker or immunocytochemical studies to be of B‐cell origin, the histopathologic features were distinctive and were characterized by a pattern of infiltration which was nodular and diffuse in both the splenic red pulp and the bone marrow, whereas involvement of the lymph nodes was pseudonodular. In one patient in whom the prolymphocytes had cytochemical characteristics suggestive of T‐cells, the distribution of the abnormal cellular proliferation in the lymph nodes was paracortical and the infiltrations of the spleen and the bone marrow were diffuse. Cancer 42:2360–2372, 1978.

Morphologic criteria for the differentiation of follicular lymphoma from florid reactive follicular hyperplasia: a study of 80 cases.

A study was made of 80 patients whose lymph nodes were characterized by the presence of follicles throughout the lymph node. The patients were divided into two groups on the basis of the clinical follow‐up information. The first group consisted of 20 patients who were alive and disease‐free at the end of five years without therapy. The patients in this group were classified as having florid reactive follicular hyperplasia (FRFH). The second group of 60 patients had progressive, recurrent, clinically active disease and were classified as having follicular lymphoma (FL). Forty‐six were dead of active disease, and 14 were alive with disease. Various morphologic parameters were evaluated at low and high magnification, and statistical comparisons were made between FRFH and FL. Although several criteria were helpful in distinguishing FRFH from FL, the single most valuable criterion was the type of pattern encountered. Follicles of variable size and shape lying adjacent to each other throughout the lymph node without or with very little intervening tissue are diagnostic of FL. This pattern was evident in 85% of the FL cases, but was not observed in any of the cases of FRFH.

Follicular (nodular) lymphoma during the first two decades of life: A clinicopathologic study of 12 patients

Twelve patients who developed non‐Hodgkins lymphoma with a follicular pattern during the first two decades of life were studied. Eight had the poorly differentiated lymphocytic type; the remaining four had the “histiocytic” type. Eleven of the 12 patients were male. Nine were asymptomatic, and eight had lymphadenopathy in the head and neck region. Comparison of ages revealed the extent of disease tended to be localized (Stages I and II) in the pediatric (<16 years old) patients (83%) and generalized in the adolescent‐young adult (16–19 years old) patients (83%). Often patients treated with chemotherapy and/or radiotherapy, eight achieved complete remissions that lasted 3–58 months (median, 17.5 months). Five are still in remission; three have relapsed. Seven are alive 12–120 months from diagnosis (median, 48 months); six have no clinical evidence of disease. The remaining five patients died two to 164 months after diagnosis (median, 13 months). Three of the four patients who died with lymphoma had diffuse “histiocytic” lymphoma demonstrated at autopsy examination. Poor prognostic factors included 1) failure to achieve a complete remission following initial therapy; 2) extranodal disease (with the exception of the poorly differentiated lymphocytic type involving the spleen and liver); 3) development of diffuse “histiocytic” lymphoma. Follicular lymphoma occurring in the second decade of life has a biologic behavior similar to follicular lymphoma in adults.

Hodgkin's disease, lymphocyte depletion type: a clinicopathologic study of 39 patients.

A clinicopathologic analysis of 39 patients with Hodgkins disease of the lymphocyte depletion type (LDHD) confirmed that this is the most aggressive form of the disease, but that it does not necessarily have a rapidly fatal course. The median survival in our series was 25.1 months; eight patients (21%) survived four years or longer. There were no clinical or survival differences between the reticular and the diffuse fibrosis subtypes. The majority (74%) of the patients were males. The median age was 48 years. Most patients in both groups presented with constitutional symptoms, peripheral lymph node enlargement and splenic or hepatic enlargement, or both. The diagnosis was established on a peripheral lymph node in 81% of the cases. Mediastinal involvement was present in 36% of the cases and was a favorable prognostic feature. Anemia and lymphocytopenia were common at presentation. Autopsy in 14 patients revealed involvement of lymph nodes in every case, liver and bone marrow in 13 cases and the spleen in all (11) cases which had not undergone prior splenectomy as part of a staging laparotomy. There was a high incidence of usually noncontiguous lung involvement. Our data indicate that despite the advanced stage of disease at presentation patients with LDHD can be salvaged with chemotherapy. They do not suggest that LDHD represents a distinct clini‐copathologic entity.

The demonstration of terminal deoxynucleotidyl transferase on frozen tissue sections and smears by the avidin-biotin complex (ABC) method

A method using avidin-biotin complex (ABC) to detect the presence of the enzyme terminal deoxynucleotidyl transferase (TdT) is described and compared with a proven indirect immunofluorescence method. The material studied consisted of: (1) peripheral blood and bone marrow smears from 17 patients with leukemia (ALL, 8; CLL, 3; AML, 6), six normal controls, one T-ALL cell line, and (2) frozen tissue sections from four patients with lymphoblastic lymphoma (LL), two patients with nodular poorly differentiated lymphocytic lymphoma (NLPD), two patients with reactive follicular hyperplasia (RFH) and one calf thymus. The slides from patients with ALL had from 30 to 90% cells with nuclear positively by the ABC technique. Slides from patients with CLL were negative, as were the normal peripheral blood smears. Normal bone marrow smears contained less than 5% positive cells. The T-ALL cell line was 100% positive. The frozen tissue sections from the patients with LL and the calf thymus contained numerous positive cells, while all of the sections from the patients with NLPD and RFH were negative. A good correlation existed between the ABC and the indirect immunofluorescence methods. The ABC method described is both more specific and more sensitive than the previously described techniques in detecting TdT in tissue sections and smear preparations.