Prem Khilanani

T-cell lymphomas of large cell type. A variety of malignant lymphomas: "histiocytic" and mixed lymphocytic-"histiocytic".

Clinical and morphologic features of seven T‐cell lymphomas of the large cell type are described. The tumors were grouped into those with irregular (3 cases) and those with round and regular nuclei (4 cases). In both groups, variation in cell size, numerous histiocytes and vessels, and many mitoses were distinguishing features. In only 1 case in the round and regular nucleus group was there relatively little variation in cell size and a paucity of histiocytes. Abundant polyribosomes, long strands of rough endoplasmic reticulum, and lysosomal granules were prominent electron microscopic features in both groups of tumors. The clinkal presentations and courses varied considerably, especially in patients with tumors of the round nucleus type. One patient presented initially with chronic lymphocytk leukemia, 1 with Lennerts lymphoma, another with bone marrow infiltration, and a fourth with subcutaneous tumors. Two patients with the round nucleus type are still alive one and a half and two years after the original diagnosis. Two patients died two years after the onset of symptoms. Each of the 3 patients with tumors of the irregular nucleus type had a rapid clinical course and died within ten months. Cancer 46:87–101, 1980.

Hairy cell leukemia in three siblings: An apparent HLA‐linked disease

In a sibship of 11 adults the HLA haplotype A1, B7 occurred in four brothers, three of whom developed hairy cell leukemia (HCL) within a seven‐year period. While this haplotype does not appear to occur with increased frequency in random cases of HCL, the fact that three cases of this uncommon and unique disorder occurred within one family and affected siblings with a common haplotype strongly suggests a genetic linkage.

Evaluation of two plasma fucosyltransferases as marker enzymes in non-Hodgkin's lymphoma.

Levels of two fucosyltransferases were measured in plasmas of patients with non‐Hodgkins lymphoma at different phases of the disease. The level of a GDP‐fucose: galactoside fucosyltransferase (EC 2.4.1.69) was found elevated in nonresponding patients and was correlated with estimated tumor burden. Enzyme levels in the normal range were found in patients in remission, maintained on chemotherapy, or unmaintained. The plasma level of a GDP‐fucose: N‐acetylglucosaminide fucosyltransferase (EC 2.4.1.68) was elevated in all individuals receiving drug therapy regardless of disease status, but returned to normal levels during unmaintained remissions.

Serum copper levels in non‐Hodgkin's lymphoma

Levels of serum copper in 34 patients with adult non‐Hodgkins lymphoma at different phases of the disease have been studied. All of the patients were evaluated with complete blood counts, sedimentation rate, gallium scintigraphy, liver and bone marrow biopsies, lymph node biopsy, and laparoscopy. The level of serum copper was significantly elevated in non‐responding or relapsing patients (mean 191.06 μg/dl), and correlated with the estimated tumor burden. Serum copper levels within normal range were found in patients in complete remission (mean 114.76 μg/dl). Age‐ and sex‐matched normal controls also showed serum copper levels within normal range (mean 112.81 μg/dl). It is proposed that serial measurements of serum copper level may be of use in: (1) monitoring the remission status of patients with non‐Hodgkins lymphoma, (2) detecting early relapse of non‐Hodgkins lymphoma, and (3) contrary to previous reports by Hrgovcic et al.,5 the level of serum copper seems to be related to the disease activity of histiocytic lymphoma.

Comparison of Circulating Colony-Forming Cells in Chronic Granulocytic Leukemia and Leukemoid Reaction

In vitro culture studies of peripheral blood leukocytes using semi-solid media from 8 patients with chronic granulocytic leukemia (CGL) and 5 patients with granulocytic leukemoid reaction were performed. A markedly increased number of circulating colony-forming units were present in patients with CGL (mean 343 +/- 47) as opposed to those having granulocytic leukemoid reaction (mean 7.0 +/- 4). The colony size was larger in CGL than in granulocytic leukemoid reaction or in normal peripheral blood.

Book Review / Announcements

Sydney E. Salmona Clinics in Haematology, vol. 11, No. 1 Myeloma and Related Disorders Saunders, Eastbourne 1982 VII + 238 pp.; E 10.75, bound ISSN 0308-2261 Within the 238 pages of this very legible text the authors have presented a broad, yet concise review of the current concepts of myeloma in the clinic and in the laboratory. The clinician concerned with the treatment of myeloma patients is provided with guidelines in distinguishing between disease categories requiring systemic treatment, the localized and extramedullary plasmacytomas best treated locally and those with monoclonal gammopathy of undetermined significance (MGUS) probably best not treated at all initially. The prognosis and therapeutic results to be expected for these various types of presentation are taken from the authors own experience and cooperative group studies. Insight into the future prospects of treatment are given not only for currently available cytostatic agents, but also for interferon as well as in vitro cloning and chemosensitivity. Such prospects are also hinted at in the chapter on immunoregulato-ry circuits in myeloma. Those seeking fundamental information regarding the pathogenic mechanisms involved in the frequent complications of this disease such as infection and humoral immunoinsufficiency, hypercalcemia, and renal insufficiency will be rewarded. The chapter on amyloidosis depicts this disease in its various forms as to clinical presentation, diagnosis, laboratory findings, and therapeutic possibilities. Last but not least, the chapter on plasma cell neoplasms and acute leukemia places the connection of these two entities in its proper perspective according to present-day knowledge and experience. The possible role of cytostatic treatment in this relationship, either direct or indirect, is discussed in detail. This book provides the student of this disease in any or all of its aspects with enjoyable and informa tive reading. The ample list of references with each chapter should more than satisfy all readers requir ing more detail. R. Sonntag, Bern Buenos Aires, Argentina, September 1-7, 1984 President: Prof. L.J. Bergna

Levels of Two Plasma Fucosyltransferases as an Index of Disease Status in Patients with Acute Myelogenous Leukemia

Publisher Summary It has been found that patients with acute myelogenous leukemias have two plasma enzymes as pertinent markers of marrow status. The level of a GDP-fucose:galactoside fucosyltransferase was found correlated with the proportion of marrow blasts, while the level of a GDP-fucose:N-acetylglucosaminide fucosyltransferase was elevated during regeneration of a normal marrow population following drug therapy. GDP-fucose:galactoside fucosyltransferase (Fuc→Gal) activity was measured with GDP-[14C] fucose and an asialo-fetuin (aSF) acceptor. The assay method for these two enzymes has been described and utilizes the preferential inhibition of the (Fuc→Gal) enzyme. During drug-induced remissions, the level of plasma enzyme activity fell to control levels. Although the plasma level of the (Fuc→GlcNAc) fucosyltransferase was not elevated above control in untreated or nonresponding patients, this level rose substantially during drug induced remissions. This chapter also explores the results of another study that was carried out and reported a fall in fucosyltransferase activity in plasma of patients with leukemia. This study was carried out with a low mol. wt. fucose acceptor.