Acute myocardial infarction associated with high dose intravenous immunoglobulin infusion for autoimmune disorders. A study of four cases.

OBJECTIVE: To report on four patients with autoimmune disorders who developed acute myocardial infarction (MI) during or soon after treatment with high dose intravenous immunoglobulins (IVIG) and to determine the clinical profile of patients prone to this complication. METHODS: The clinical history of the four patients is reported with details concerning age, sex, indication for IVIG treatment, risk factors, timing of the MI and outcome. The relevant medical literature has been reviewed. RESULTS: The patients, three men and one woman, aged 42-67, received IVIG treatment for different autoimmune disorders. All had a history of atherosclerosis or previous risk factors such as hypertension, stroke, hyperlipidaemia and obesity. Two of the patients suffered a MI after the first infusion of IVIG while the others-after the 5th and 15th pulses. MI occurred during the infusion in two patients and after a few days in the others. All the patients recovered from the acute event. These observations are in concert with sporadic cases of IVIG related thrombosis reported in the medical literature. CONCLUSION: In patients with vascular risk factors such as old age, hypertension, history of stroke or coronary artery disease, the possibility of IVIG related vascular complications should be considered and IVIG prescribed with a cautious reweighted risk/benefit consideration.

Translocation (1;20)(q32;q13.3) in myelofibrosis following polycythemia vera.

We report a novel chromosomal translocation (1;20)(q32;q13.3) in a patient with myelofibrosis following polycythemia vera. This 73-year-old woman developed myelofibrosis 6 years after the initial diagnosis of polycythemia vera (PV). The course of PV was uneventful. Subsequent to the diagnosis, the patient was treated with phlebotomy and low doses of hydroxyurea for 4 years. No therapy was delivered during the remaining 2 years. A bone-marrow biopsy and a karyotype analysis performed because of evolving anemia demonstrated myelofibrosis and a chromosomal aberration-t(1;20)(q32;q13.3). Aberrations in chromosomes 1 and 20 have been reported in myeloproliferative disorders, but a t(1;20) translocation has not been reported. Because a karyotype analysis was not performed at the time PV was diagnosed, whether this translocation is linked to the primary disease (PV) or to the transition to myelofibrosis is not known.

Pure red cell aplasia responsive to interferon-alpha in a patient with hepatitis C virus infection.

A 51-year-old man presented with severe anemia, mild splenomegaly and elevated serum aspartate aminotransferase and serum alanine aminotransferase levels. The bone marrow findings were consistent with pure red cell aplasia (PRCA) with a 'maturation arrest' at the level of pronormoblast. The patient has been transfusion-dependent for 8 months. Following diagnosis of chronic active hepatitis due to hepatitis C virus (HCV), therapy with interferon-alpha was initiated. Two weeks later, the hemoglobin level stabilized, and he has not required any transfusion ever since. In spite of ongoing HCV viremia, cessation of interferon therapy, and deterioration of the liver function tests, the patient, followed for 2 years, maintains a high-normal hemoglobin level. To the best of our knowledge, this is the first report of prolonged PRCA corrected by interferon-alpha therapy, with or without an ongoing HCV infection. We speculate that the 'maturation arrest' of the erythroid lineage seen in the bone marrow was the result of an immune mechanism, possibly induced by the HCV, and that the elimination of this mechanism, rather than the elimination of the HCV, provided the opportunity for regeneration of erythropoiesis.

Deletion of the long arm of chromosome 7 in myelomonocytic leukemia with bone marrow eosinophilia.

We report a 62-year-old man with acute myelomonocytic leukemia with bone marrow eosinophilia (M4Eo), and a deletion of the long arm of chromosome 7. The patient presented with pancytopenia, which shortly after evolved to overt leukemia. There was no response to the daunorubicin-cytosine arabinoside (Ara-C) regimen, and a remission achieved with amsacrine (AMSA)-Ara-C lasted only for a short time. On relapse, a peculiar skin rash accompanied the hematologic picture. While ANLL with chromosome 7 abnormalities usually carries adverse prognosis, patients with M4Eo (which is usually associated with chromosome 16 abnormalities) do better. The patient described here examplifies that M4Eo may be associated with del(7)(q22), and that it is the chromosomal abnormality rather than the type of leukemia that might determine the clinical outcome.

Flare-up of squamous cell carcinoma of the skin following fludarabine therapy for chronic lymphocytic leukemia.

We present a 72-year-old patient with chronic lymphocytic leukemia (CLL). About a year following therapy with chlorambucil and prednisone, he suffered from anemia, thrombocytopenia and organomegaly. The patient received fludarabine with a favorable response. Concomitantly with the clinical improvement of the CLL there was a remarkable flare-up of scalp squamous cell carcinoma (SCC) lesions, initially noted 4 years previously. The lesions were multiple and grew rapidly. Fludarabine depresses the T lymphocyte population, cells that play a pivotal role in the regression of the SCC. We suggest, that the flare-up and exacerbation of the SCC lesions of the patient were triggered by the fludarabine therapy.