A patient with immunoblastic lymphadenopathy (IL) had an unusual course of illness, with frequent episodes, over a 20-month period, of chills, fever, abdominal pain, hepatosplenomegaly and weight loss. The episodes were short-lived and many resolved spontaneously. Eventually generalized lymphadenopathy and profound monoclonal lgG gammopathy developed, with atypical mononuclear cells In the peripheral blood and increased numbers of plasmacytoid cells in the bone marrow. Lymph node biopsy revealed the morphologic triad typical of IL: proliferation of immunoblasts, proliferation of small blood vessels and the deposit of an amorphous acidophilic material in the vascular walls and the interstitium. Up to October 1976 110 cases had been reported of this disorder, first described 3 years ago, which indicates that IL is not rare. Remissions have occurred spontaneously and after steroid therapy or chemotherapy or both. However, death has been reported in almost 50/o of the cases, and the best approach to therapy remains to be determined. Une patiente atteinte de lymphadenopathie immunoblastique (LI) avait une 6volution inusit6e de Ia maladie, evolution caract6risee par de nombreux 6pisodes de frissons, fievre, douleurs abdominales, h.patosplenomegaiie et perte de poids, le tout sur une periode de 20 mois. Ces episodes 6talent brefs et plusleurs ont eu une r6solution spontan6e. Par Ia suite une lymphadenopathie generalisee et une gammopathie monoclonale lgG ont d6veloppe, avec des celluies mononucleaires atypiques dans le sang peripherique et un nombre 61ev6 d'6l6ments plasmocytoides dans Ia moelle osseuse. Une biopsie ganglionnaire a d6montr6 Ia triade morphologique classique de Ia LI, soit une prolif6ration immunoblastique, une prolif6ration de vaisseaux sanguins petits et Ia deposition d'un materiel acidophile amorphe au niveau des parois vasculaires et de i'espace interstitiel. Depuis Ia premiere description de From the departments of medicine, hematology and pathology, Jewish General Hospital and McGill University, Montreal Reprint requests to: Dr. A.A. Cooperberg, Jewish General Hospital, 3755 Cote Ste. Catherine Rd., Montreal, PQ H3T 1E2

cette entite il y a 3 ans, 110 cas ont ete publies (jusqu'a octobre 1976), ce qui suggere quo Ia LI n'est pas rare. Des remissions spontanees et des remissions apres corticotherapie ou chimiotherapie ou les deux sont decrites. Cependant, Ia mort survient dans 5001o des cas, et Ia meilleure approche therapeutique reste a determiner. Immunoblastic lymphadenopathy (IL) is a recently described lymphoma-like disorder with characteristic morphologic features in biopsied lymph nodes.1'2 These features consist of diffuse obliteration of the lymph node architecture with immunoblasts, plasmacytoid immunoblasts, plasma cells and lymphocytes with intermediate forms. The cellular infiltrate involves the capsule and perinodal tissues as well. There is proliferation of arborizing small blood vessels with endothelial hyperplasia and an interstitial deposit of amorphous acidophilic material. Occasionally eosinophils and Reed-Steinberg-like cells have also been noted.2 Immunoglobulin abnormalities are common and, because of this, Frizzera, Moran and Rappaport1 have called the condition angioimmunoblastic lymphadenopathy with dysproteinemia. Clinically IL is associated, in an elderly patient, with recurrent episodes of fever, weakness, night sweats and weight loss. Hepatosplenomegaly and generalized lymphadenopathy are usually present. This combination of clinical and histologic findings has often led to an erroneous diagnosis of Hodgkin's disease,2 malignant lymphoma3 or plasmacytoma.4 Because most of these cases were originally recognized by two consultant pathologists1'2 reviewing biopsied or postmortem material, much of the clinical and laboratory information was either incomplete or not available;1 their reviews encompass 56 cases. Detailed reports of individual patients, few of which have been published, are particularly important now to gain a better understanding of the clinical and laboratory features as well as the natural history of this disease. In this issue of the Journal (page 41) Averback, Salama and Moinuddin describe in detail a patient with Hashimoto's disease and IL, a previously unreported association. Our patient presented with a number of unusual features and was observed







diagnostic findings appeared. Case report Clinical history An 83-year-old woman was admitted to hospital in January 1975 because of abdominal pain and dehydration. She had had 13 previous admissions over an 18-month period to December 1974. On each occasion she presented to the emergency room because of a sudden onset of high fever (temperature, 40'C), chills, abdominal or chest pains, stupor and dehydration. Usually a pansystolic murmur was heard and the spleen was palpable 2 to 3 cm below the left costal margin. The liver edge was felt on only one or two occasions and there was never any lymphadenopathy. She had persistent hemolytic anemia, with a negative Coombs' test, moderate thrombocytopenia (Table I) and a prolonged prothrombin time. Cultures were repeatedly negative except during the occasional urinary tract infection with Escherichia ccli. She usually responded within 24 to 48 hours to intravenous therapy with or without antibiotics. Tests for rheumatoid factor, cryoglobulins and cold agglutinins were repeatedly negative. Mild polyclonal gammopathy was evident on a number of occasions with both immunoglobulin and protein electrophoretic studies (Table II). In 1972 a tuberculin test had been positive and a histoplasmin test negative. During the year and a half of recurrent fever the patient received digoxin, furosemide, potassium, diazepam, dimenhydrinate, acetaminophen, chloramphenicol, gentamicin, penicillin, sulfisoxazole, morphine, pentobarbital, meperidine, ampicillin and acetylsalicylic acid. On the 14th admission she had no fever but there were generalized firm, matted, enlarged lymph nodes and the liver and spleen were both palpable 5 cm below the costal margins. The blood now contained a few abnormal large mononuclear cells resembling atypical lymphocytes. Serum and erythrocyte folate values, which had been repeatedly normal in the past, were now low (Table 1). The concentration of total serum proteins was now greatly increased and a monoclonal IgO gammopathy was detected (Table II). The test for urinary Bence Jones protein was negative. There was now a negative reaction to tuberculin, histoplasmin, mumps antigen and trichophytin, but the reaction to Candida albicans antigen was positive. Clinical progress A lymphoma with a gammopathy was strongly suspected at this time and therapy with cyclophosphamide (400 mg/d for 4

CMA JOURNAL/JULY 9, 1977/VOL. 117 53

Table 1-Hematologic data* in patient with immunoblastic lymphadenopathy

Date May 1973 Feb. 1974 Sept. 1974 Jan. 11, 1975 Jan. 20, 1975 Sept. 1975

Cell counts (X 10./L) RetiHemoglobin, culocytes, g/dL Platelets Leukocytes (14 2) (300 100) (5 - 10) Differential (0.5 - 1.5) 10.3 172 4.5 N 1.0 9.8



9.0 10.1 5.2 8.8

72 67 21 70

13.0 9.6 2.1 2.7

Serum folate, ng/mL (3 - 12) 11.8

RBC Serum folate, Serum B12, haptoglobin, Bone ng/mL pg/mi mg/dL marrow (275 - 700) (180 - 900) (50 - 100) 136 1897 85 No abnormal cells N 7.2 3.8 419 626 22 No abnormal cells LE 10.0 25 LEt 1.0 224 416 Abnormal. N 1.2§ N 1.8 No abnormal cells N 2.6 6.4 982 12 = normal; LE = leukoerythroblastic, or presence of both immature granulocytes and normoblasts.

Sept. 1976 10.3 96 2.0 *Normal values in parenthesis. Abbreviations: N tAtypical mononuclear cells In blood smears. lBone marrow infiltrated with plasmacytoid cells. RAfter chemotherapy.

Table ll.Results* of serum protein, gamma globulin and enzyme studies Total Gamma Complement Alk. protein, globulin,t lgA.t lgG,. lgM, (.1C), Serum Bilirubin, phos., LDH, SGOT, g/dL g/dL mg/dL mg/dL mg/dL mg/dL viscosity mg/dL mU/mi mU/mi mU/mi Date(6 - 8) (0.8. 1.6) (90. 400) (600 - 1500) (50. 200) (100. 170) (1.4 - 1.8) (

Immunoblastic lymphadenopathy: case report and literature review.

Immunoblastic lymphadenopathy: case report and literature review ARTHUR A. COOPERBERG, MD, CM, M SC, FACP, FRCP[C]; MARIE BRISSON DE CHAMPLAIN, MD; JE...
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