Ila Mirchandani

B-cell lymphomas morphologically resembling T-cell lymphomas

Seven cases of B‐cell lymphoma that morphologically resembled T‐cell lymphoma are described. These cases are of four morphologic types: atypical poorly differentiated lymphocytic lymphoma (PDLL) with convoluted nuclei, “Lennerts” lymphoma, mixed lymphocytic‐“histiocytic” lymphoma with large variation in size of abnormal cells, and “histiocytic” lymphoma with large multilobed nuclei. These cases add further support to the belief that morphologic criteria alone are not sufficient for accurate immunologic classification of the malignant lymphomas since they may represent a distinct clinicopathologic entity. Cancer 56: 1578‐1583, 1985.

Acute megakaryoblastic leukemia

Acute megakaryoblastic leukemia is a rare and rapidly fatal disorder characterized by extensive proliferation of megakaryoblasts and atypical megakaryocytes in bone marrow and extramedullary sites, thrombocytopenia and only a few blasts in peripheral blood. Three cases of this leukemia were studied morphologically, cytochemically, and electron microscopically. The leukemic blasts varied from 10–20 m̈ in diameter, had coarsely reticular nuclear chromatin, and numerous cytoplasmic projections and vacuoles. Except for intense granular PAS positivity and diffuse acid phosphatase reactivity, all of the usual cytochemical stains were negative. The blasts had no specific differentiating features identifying them as megakaryoblasts even at the ultrastructural level. In such instances demonstration of platelet peroxidase will confirm the megakaryocytic origin. All three patients in this series were men and all died within 90 days. Two patients also had other malignancies.

Lymphocytic lymphoma simulating hairy cell leukemia: A consideration of reliable and unreliable diagnostic features

Morphologic, immunologic and functional characteristics of an unusual poorly differentiated diffuse lymphocytic lymphoma closely resembling hairy cell leukemia are presented and the diagnostic features of the latter disease are reviewed. The malignant cells morphologically resembled hairy cells at the light as well as electron microscopic level. They had surface characteristics of both T and B lymphocytes, were phagocytic, and adhered to glass. The spleen was smooth and resembled that seen in hairy cell leukemia. Although there was a predilection for the red pulp of the spleen, there was a lack of characteristic pseudosinus formation, and areas of predominantly white pulp involvement considered characteristic of lymphocytic lymphoma were found after extensive examination.

True histiocytic lymphoma. A report of four cases

Clinical, morphologic, cytochemical, immunologic, and ultrastructural features of four cases of true histiocytic lymphoma are described. The neoplastic cells were large, ranging from 20 to 45 μ in diameter with round, folded, or convoluted nuclei, and abundant eosinophilic cytoplasm. They exhibited diffuse nonspecific esterase activity. Diffuse acid phosphatase activity was present in two cases so tested. Muramidase activity was present in half of the cases. Finely granular PAS‐positive material was seen in the cytoplasm. Methyl green‐pyronin positivity was variable. An occasional neoplastic cell showed erythropagocytosis in one case. Malignant cells either contained no cytoplasmic immunoglobulins (three cases) or had immunoglobulins of multiple classes (one case). Surface markers were studied in two cases; they were absent in one case, and were of multiple classes in another case. Ultrastructurally the neoplastic cells had lysosomal granules in three cases so examined, and phagolysosomes, phagocytized material and residual bodies in one of three cases so studied. Patients ranged in age from 28 to 60 years. Two patients had extralymphatic tumors. Survival of more than 5 years was seen in one patient.

Sézary syndrome with a 14:14 (q12:q31) translocation

The Sézary syndrome was diagnosed in a 71‐year‐old black woman with erythroderma, generalized lymphadenopathy and hepatosplenomegaly. The laboratory data revealed a white blood cell count of 65,000 mm3 with 81% lymphocytes, the majority having an indented or a cerebriform nucleus. The skin biopsy, the lymph node biopsy and immunologic surface marker studies confirmed the diagnosis of Sézary syndrome. The cytogenetic studies of the bone marrow and the peripheral blood revealed a (14:14) (q12:q31) translocation present, consistently in majority of the lymphocytes. This translocation is considered characteristic of ataxia‐telangiectasia and, to our knowledge, has not been previously described in Sézary syndrome.

Sudden infant death syndrome: measurement of total and specific serum immunoglobulin E (IgE).

Postmortem evaluation of total and specific serum immunoglobulin E (IgE) antibody levels by the paper radio immuno sorbent test (PRIST) and radio allergo sorbent test (RAST), respectively, revealed that there was no significant elevations in total circulating IgE or in specific IgE antibodies to house dust, Dermatophagoides farinae (house dust mite), Alterarnia tenuis (mold), or milk proteins for sudden infant death syndrome (SIDS) victims when compared to a control group.

Hand mirror cell leukemia—immunologic and ultrastructural studies

Acute lymphoblastic leukemia (ALL) with hand mirror cell (HMC) variant was diagnosed in a 26‐year‐old black man in May 1978. Hemoglobin was 3.6 g/dl; the platelet count was 19.0 × 109/1; leukocyte count was 8.4 × 109/1 with 40% blasts, 66% of which had HMC appearance. Cytochemical studies, terminal deoxynucleotidyl transferase level, and immunologic marker studies indicated a non‐T/non‐B lymphoblastic origin of the leukemic population. Electron microscopic studies confirmed the hand mirror appearance. Mitochondria were more numerous in these cells compared with other lymphoid cells. Cytogenetic studies showed a 46XY karyotype. Our studies confirmed the previous studies reported by Stass, et al.22 of lymphoblastic origin of HMC leukemia. The patient responded to treatment with vincristine, prednisone and L‐asparaginase and went into complete remission. It appears that this morphologic variant of ALL does exist and is not an artifact.

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