Search

Fanconi’s anemia is an inherited disorder characterized by multiple congenital abnormalities, chromosome anomalies and irreversible aplastic anemia. Patients, and possibly family members, have been noted to have an increased incidence of leukemia and other tumors. A family with two affected male children, one of whom developed acute myelomonocytic leukemia, is described. Chromosome preparations in both parents showed abnormalities, a previously unreported finding. Fibroblast cultures from both parents and one brother inoculated with Simian Virus 40, a known oncogenic agent, developed increased numbers of transformed colonies, compared with normal control cultures similarly inoculated. The presence of chromosome abnormalities and cellular susceptibility to oncogenic agents appear to be related to an increased risk of malignancy.

Leukocyte alkaline phosphatase activity has been noted to be different in men and women. The mean leukocyte alkaline phosphatase activity for 74 normal men, aged 19 to 60 years, was 23 mg. of phosphorus per 1010 polvmorphonuclear leukocytes per hour. The corresponding mean value for 75 normal young women, age 19-48 years, was 35 (p < .001). No significant differences between boys and girls occurred until the time of puberty. After the menopause, the values for women approached the values for men. Women treated with androgens had lower leukocyte alkaline phosphatase activity than did control women. These results suggest that androgenic hormones inhibit this enzyme, and that other, as yet undefined endocrine influences, also affect its level of activity. In vitro tests with various concentrations of androgens and estrogens failed to provide conclusive evidence of direct effect on leukocytes although some degree of direct inhibition by androgens was suggested. Studies using saponin to effect enzyme release from leukocyte granules did not demonstrate whether the differences between men and women are differences of enzyme release or of content of leukocyte alkaline phosphatase.

Leukocyte alkaline phosphatase was measured by a biochemical method in a group of 58 mongolian idiots. The mean activity found in the 41 children less than 10 years of age was equivalent to 89 mg. of phosphorus per 1010 neutrophilic leukocytes per hour. This was compared to the value of 66 mg. of phosphorus per 1010 neutrophilic leukocytes per hour obtained in a group of 41 control children in the same age group (after correction of the control mean to the same mean age). The difference is significant at the 0.5 per cent level of confidence. This difference was interpreted as confirming (but not proving) the hypothesis that leukocyte alkaline phosphatase formation is controlled by a gene on chromosome number 21—the chromosome for which mongolian idiots are trisomic. The hypothesis arose because of the known deficiency of this enzyme in the leukocytes of patients with chronic granulocytic leukemia, and the known partial deletion of chromosome 21 in this disease. This finding should provide a stimulus to further investigations into the content of alkaline phosphatase in leukocytes, possible polymorphism of leukocyte alkaline phosphatases, linkages with other inherited traits, and relationships between leukocyte and other tissue phosphatases.

Dying leukocytes suspended in normal serum tend to agglutinate spontaneously into a firm "clot." This phenomenon is associated with cell lysis and the formation of long fibrous strands of DNA-protein. It seems likely that nuclear DNA-protein actually combines in some way with a fraction of the serum proteins (possibly mucoprotein). Such leukocyte agglutination is inhibited by serum containing the L.E. cell factor. LAI is associated with nuclear changes which are characteristic of the L.E. cell phenomenon, and it is most probable that it is an expression of the L.E. cell phenomenon. An understanding of the chemistry of LAI may lead to useful information as to the fundamental mechanism of the L.E. cell phenomenon. Without such understanding, LAI has been used to obtain collateral information concerning the L.E. cell phenomenon: namely, to rule out any important role for DNA-ase, and to confirm the importance of blood platelets. It has also been used to prepare "pure cultures" of L.E. bodies for cytochemical study. On a clinical level, LAI may prove a useful adjunct to the standard L.E. cell tests. However, its lower level of sensitivity and occasional "false positive" reactions make it inadequate as a primary test in systemic lupus.

INTRODUCTION This is a report which demonstrates how the administration of a variety of drugs can produce a clinical syndrome indistinguishable from systemic lupus erythematosus (SLE). Among these drugs, the best known is hydralazine.1 This agent, when used continuously for several months in the treatment of hypertension, will usually induce a clinical syndrome resembling SLE.2 Similar syndromes have recently been reported when anticonvulsant drugs have been administered.3-6 Apparent induction of the lupus erythematosus (LE) cell phenomenon by antituberculous therapy has recently been reported in one patient.7 Another publication 8 states that aminosalicylic acid apparently also induced the LE cell phenomenon. Review of the original publication,9 quoted by Simpson and Walker, fails to substantiate this report.The present report concerns a patient who developed a systemic lupus-like syndrome while on antituberculous therapy. Temporal relationships and clinical features of this case make it appear likely that the

Cells from bone marrow aspirates and liver, kidney and spleen imprints from patients with leukemia and other nonleukemic hematological and non-hematological disorders were tested for reactivity with murine leukemia (Rauscher) virus antibody using the technique of immunofluorescence. The cells of all eleven patients with acute lymphoblastic leukemia and four out of five patients with chronic lymphatic leukemia reacted strongly. In only six of the 15 patients with myelogenous leukemia and approximately one-third of the 47 patients with miscellaneous disorders (Table 1) was strong immunofluorescence demonstrated.

Systemic lupus erythematosus (S.L.E.) is associated with striking plasma protein changes, among which hypergammaglobulinemia is usually prominent. The purpose of reporting this case in detail is to call attention to a patient with S.L.E. associated with the rare feature of a chronically low concentration of γ-globulin. This is unique because, to the best of our knowledge, this is the first case of S.L.E. with hypogammaglobulinemia in the pediatric age group to be recorded in detail.In this report an opportunity was afforded to study the survival time of intramuscularly injected γ-globulin.This case also presented the opportunity to observe the effects of continuous corticosteroid therapy for three years and intramuscularly injected γ-globulin over a period of two years. REPORT OF CASE On May 4, 1956, this 9-year-old white girl with anorexia, low-grade fever of six weeks' duration, and a pruritic erythematous facial rash of four days' duration was admitted to

Histiocytic medullary reticulosis was first described as a clinical and pathological entity by Scott and Robb-Smith in 1939. 1 The clinical picture includes fever, wasting, lymphadenopathy, hepatosplenomegaly, and progressive pancytopenia. The outstanding morphologic feature of the disease is proliferation of histiocytes throughout the lymphoreticular system and frequent erythrophagocytosis. Most patients die within six months after onset of disease; the average duration before death is 15 weeks. The disease occurs in adults between the ages of 20 and 70 years and there is a 3:1 male predominance.2,3The case reported here was unusual in that the patient manifested severe thrombocytopenia which was thought to be due to massive platelet phagocytosis by abnormal histiocytes. He died of massive gastrointestinal hemorrhage. PATIENT SUMMARY A 21-year-old white man presented on Jan 13, 1968, because of six weeks of anorexia, weakness, weight loss, daily fevers to 104 F (40 C), and bilateral cervical

A factor derived from blood platelets has been shown to be necessary for the activation of the L. E. cell factor. This L. E. cell cofactor may be identical with the platelet thromboplastic factor. The relation of these findings to occurrence of the L. E. cell phenomenon in vivo and to the occurrence of a coagulation defect in systemic L. E. has been discussed.

SYNDROMES with clinical, laboratory, and anatomical features identical with those of systemic lupus erythematosus have been reported after administration of certain drugs. A list of agents which have been implicated is contained in Table 1. The drugs listed do not seem to have a single common denominator either chemically or pharmacologically. With the exception of penicillin, they do have in common the feature that in each case, systemic lupus erythematosus has followed use of the drug in high dosages for periods in excess of two months.The present study represents an attempt to determine what importance prior intensive drug therapy might have in the development of signs and symptoms of systemic lupus erythematosus. METHODS AND MATERIALS GENERAL PLAN. —Case records of patients diagnosed clinically as having systemic lupus erythematosus SLE were examined from the point of view of symptoms, diagnosis, and therapy administered before onset of systemic lupus erythematosus.Records

Anemic crises occur during the course of chronic hemolytic anemias, both congenital1-3 and acquired.4-6 These crises, due to temporary cessation of red cell production, are usually related to acute viral infections. Acute erythroblastopenia has also been reported in relation to treatment with various drugs, including chloramphenicol.7-10The cases reported here are noteworthy for 4 reasons. First, they represent examples of anemic crisis occurring in the course of serious constitutional illnesses in which hemolysis did not seem to be important prior to the appearance of the crisis. Second, all 4 patients studied were receiving chloramphenicol at the time of development of erythroblastopenia. Third, striking "toxic" changes were noted in bone marrow cells at the height of the crisis in all 4 cases; changes of the type to be described have not been emphasized in most previous reports. Finally, in 1 of the 4 cases evidence has been obtained

Laboratory studies in systemic lupus erythematosus, as in many other diseases, are important in two ways: first, to establish a diagnosis and aid in the treatment of individual patients; second, to help in understanding the development of the disease process. This discussion will be limited to the more general aspects of laboratory findings in this disease.The description of the L. E. cell by Hargraves, Richmond, and Morton1 in 1948 was of foremost importance because it established a simple and reliable diagnostic test for a disease which, until then, had been extremely difficult to diagnose. At the same time the L. E. cell test made two other claims for interest. First, because atypical as well as typical cases could now be diagnosed with some certainty, the importance of other abnormal findings and their relationship to one another could be assessed. A yardstick was

Leukocyte alkaline phosphatase was measured by a biochemical method in a group of 58 mongolian idiots. The mean activity found in the 41 children less than 10 years of age was equivalent to 89 mg. of phosphorus per 1010neutrophilic leukocytes per hour. This was compared to the value of 66 mg. of phosphorus per 1010neutrophilic leukocytes per hour obtained in a group of 41 control children in the same age group (after correction of the control mean to the same mean age). The difference is significant at the 0.5 per cent level of confidence.