Allen D. Schwartz

Cat-scratch disease simulating malignant lymphoma.

A six‐year‐old girl with induration, swelling and discoloration of the lower eyelid, a temporal mass, preauricular adenopathy and enlarged parotid gland, underwent biopsy. She was initially diagnosed as having a malignant disorder of histiocytic origin. All lesions resolved without therapy. Further evaluation revealed that the child had oculoglandular cat‐scratch disease. Cat‐scratch disease should be added to the list of nonmalignant disorders which may simulate a malignant neoplasm in its clinical and histologic appearance. Recognition of this fact is important in order to avoid erroneous diagnosis, unnecessary procedures and hazardous therapy.

A fatal myeloproliferative syndrome in a family with thrombocytopenia and platelet dysfunction.

Three siblings with a lifelong history of a bleeding disorder and thrombocytopenia died from a myeloproliferative disease. In 2, the terminal event resembled juvenile chronic myelogenous leukemia, and in the third, the diagnosis was acute monocytic leukemia. A family study revealed that the mother and 5 other siblings had a variety of hematologic abnormalities. These included chronic thrombocytopenia, abnormal platelet function, elevated concentrations of HgbF or serum vitamin B12, and low leukocyte alkaline phosphatase (LAP) scores either singly or in combination. At the time of study, none had evidence of malignancy. Members of this family have a myeloproliferative disorder that has the potential for terminating in nonlymphocytic leukemia, a combination of events which appears not to have been reported previously.

67Ga scintigraphy in granulocytic sarcoma

Several granulocytic sarcomas (chloromas) developed in a patient with acute myelogenous leukemia while in hematologic remission. A positive diagnosis of the symptomatic lesion was made by means of open biopsy examination. The other lesions, which were unsuspected, were detected with a 67Ga‐citrate scan. Subsequent 67Ga‐citrate scans indicated a favorable response to treatment. The incidence and significance of silent granulocytic sarcomas in patients in hematologic remission is not known. Documentation of such lesions might prove valuable for diagnosing extramedullary relapse or for delivering intensive local therapy.

Elevation of Pyrimidine Enzyme Activities in the RBC of Patients with Congenital Hypoplastic Anaemia and their Parents

Summary. The activities of orotate phosphoribosyl transferase (OPRT) and orotidine monophosphate decarboxylase (ODC) were significantly elevated (P<0.001) in erythrocytes (RBC) from five patients with prednisone‐responsive congenital hypoplastic anaemia (CHA). (OPRT: patients=10.1–64.2 nmol/h/109 RBC; controls=2.8·0.3 (mean×SEM, n=37); ODC: patients=30–124 nmol/h/109 RBC; controls=10.2×0.7 (mean×SEM, n=37).) Two patients had a less pronounced, but significant, increase of aspartate transcarbamylase activity and three patients had marginal increases of dihydroorotase activity. Dihydroorotate dehydrogenase activity was not detected in any CHA patient or control. In one patient prior to prednisone therapy, the OPRT and ODT activities were elevated 10‐fold and remained elevated 3‐fold after 16 months of therapy. An elevated enzyme pattern similar to that of RBC from CHA patients was observed in three parents of three CHA patients, but not in three parents of two other CHA patients. The activities of all five pyrimidine enzymes were normal for one patient with transient erythroblastopenia of childhood. In contrast, the activities of all the pyrimidine biosynthetic enzymes were elevated in blood from patients with a young RBC population: sickle cell anaemia, sickle‐β‐thalassaemia, hereditary spherocytosis, and DiGuglielmo syndrome and from the newborn. It is postulated that factors which affect the activities of pyrimidine enzymes in CHA may also result in diminished erythropoiesis.

The effects of four commonly used drugs on platelet function

A number of commonly used drugs have been reportd to inter