A. Weinberger

Bleeding Due to Thrombocytopenia in Acute Leukemias and Reevaluation of the Prophylactic Platelet Transfusion Policy

Prophylactic platelet administration is indicated at counts below 20×109/1. The bleeding tendency and severity were compared between thrombocytopenic patients with acute-lymphocytic leukemia (ALL) and acute non-lymphocytic leukemia (ANLL) in the ranges of 10–20×109/1 platelets, while prophylactic platelet administration was given only below 10×109/1. The bleeding tendency for ALL was quite similar at platelet counts above or below 10×109/1. The bleeding tendency was significantly lower (p<0.001) when the platelets were above this level in ANLL patients. When the thrombocytopenia was caused by chemotherapy, the bleeding was significantly lower in both types of leukemia above 10×109/1 (p<0.05 for ALL, p<0.001 for ANLL) as compared with lower counts. When the thrombocytopenia was caused by leukemia, the bleeding tendency was similar in both types of leukemia and at all platelet counts (below 20×109/1). Fever, not associated with sepsis, augmented the bleeding severity of patients with ANLL. Stable or rising counts of platelets were associated with significantly lower bleeding tendency above 10×109/1 only in ANLL patients. The decision for prophylactic platelet administration at counts below 20×109/1 should be guided by the type of the leukemia (ALL vs. ANLL), the cause of throm-bocytopenia (chemotherapy vs. leukemia per se), the trend of the platelet counts, presence of fever and patients age (below or above 18 years). Prophylactic platelet administration can be safely postponed until the count is below 10 × 109/1 in paitents with ANLL, without fever, with chemotherapy-induced thrombocytopenia and older than 18 years, regardless of the platelet trend.

Prevalence and clinical aspects of Behcet's disease in the north of Israel.

Behcet’s disease (BD) has a higher prevalence in countries along the ancient silk route, but the actual prevalence in Israel is unknown. We evaluated the occurrence and clinical expression of BD in the northern region of Israel: in the whole population and by ethnic groups. The sample included all adult patients with BD (International Study Group criteria) treated at three medical centers in northern Israel. Patient data were collected by file review and physician survey. Relevant demographic data for the population served by the medical centers were obtained from the official Israeli authorities. A total of 112 patients were identified. The overall prevalence of BD was 15.2/100,000 and was similar in men and women. The prevalence rates among the Jewish, Arab, and Druze populations were 8.6, 26.2, and 146.4 per 100,000, respectively. Age at disease onset was similar in all ethnic groups and significantly lower in males (28.6±9.7 vs 32.9±11.3, p=0.03). There were no differences in disease manifestations by sex or ethnicity. All Druze patients were HLA-B5 positive, compared to 80.8% of the Arab patients and 72.0% of the Jewish patients. Recurrent oral ulcers in family members were more common in Arab patients (p=0.004). The BD severity index was significantly lower in Druze patients (p=0.05), mainly in males (p=0.03). This study confirms the high prevalence of BD in Israel and the variability in disease rates and expression by ethnic origin. Our findings, particularly regarding the Druze population, call for further field surveys and genetic studies.

Diagonal Ear Lobe Crease and Coronary Risk Factors

The prevalence of ear lobe crease (ELC) was determined in 421 patients with myocardial infarction (MI) and in 421 controls. A higher prevalence (p < 0.05) of ELC was found in MI patients (77 percent) in comparison to controls (40 percent), regardless of age. In addition, a higher prevalence was found in patients in whom MI was combined with diabetic retinopathy or hypertension, and in Ashkenazi Jews compared to non‐Ashkenazi Jews. Ear lobe biopsies in 12 subjects revealed tears of the elastic fibers in all subjects with ELC, and prearteriole wall thickening in subjects with MI and/or ELC, but not in the 2 subjects with neither MI nor ELC. The early appearance of ELC may imply the existence of coronary heart disease with or without coronary risk factors.

Hypouricemia, Hypercalciuria, and Decreased Bone Density: A Hereditary Syndrome

Genetically determined hypouricemia in man due to increased renal urate clearance is usually associated with additional renal tubular defects (1–3). Renal hypouricemia due to an isolated renal tubular defect has also been described but appears to be a rare condition (4,5). We report a new genetically determined syndrome in man, in which the renal hypouricemia is associated with idiopathic hypercalciuria and decreased bone density.

Plasma proteins in synovial fluids of normal human joints

In this report, we will review present knowledge of the concentrations of proteins in normal synovial fluid; we will consider the mechanisms that determine those concentrations; and we will suggest additional directions that still need to be pursued

Familial Hypouricemia Due to Isolated Renal Tubular Defect

A 37-year-old female was found to have hypouricemia (1.1-1.9 mg%) with markedly increased uric acid clearance (24.7039.5 ml/min). Uric acid excretion was only slightly affected by pyrazinamide, a drug which suppresses renal tubular uric acid secretion, and by probenecid, a drug which inhibits tubular uric acid reabsorption. The attenuated response in this subject to both drugs suggest a renal tubular defect in the proximal high capacity-high affinity uric acid reabsorption mechanism. No other renal tubular or metabolic abnormalities were detected. A survey of the family-three sisters and two brothers, revealed two similarly affected sisters. The abnormality described in this family is defined as familial renal hypouricemia due to an isolated renal tubular defect with attenuated response of uric acid clearance to probenecid and pyrazinamide.

Uncontrollable life-threatening status asthmaticus. An indicator for termination of pregnancy by cesarean section

Two pregnant women with severe status asthmaticus unresponsive to intensive medical treatment are reported. Termination of pregnancies by cesarean section was followed by a dramatic improvement within 24 h. Termination of pregnancy is indicated in life-threatening status asthmaticus.

Effect of Weather Conditions on Acute Gouty Arthritis

82 patients were retrospectively evaluated for the effects of maximal and minimal temperatures, barometric pressure, wet and dry temperatures and heat stress on the five consecutive days preceding an acute gouty attack. Maximal temperature on day four preceding the attack was higher than the monthly mean [p < 0.01], the fifth days lower than the monthly mean minimal temperature [p < 0.05], and the mean barometric pressure of the fifth day before the attack higher then the monthly mean [p < 0.02]. On day four before the acute gouty arthritic attack heat stress was significantly higher than the mean monthly heat stress [p < 0.03]. The same findings were noted between the difference of the fifth night wet and fourth night dry temperatures, which were higher than those on the day of the attack [p < 0.05 and p < 0.03 respectively]. These weather changes were not specific to a certain month. Weather changes which occur four to five days before an acute gouty attack may play a significant role in precipitating the attack.

Familial Leukopenia Among Yemenite Jews

Benign familial leukopenia was found in 75 of 200 healthy Yemenite Jews examined. The leukopenia was not a constant finding and was not associated with a tendency toward infection. HLA typing showed no significant differences in the frequency of the various HLA antigens between the subjects with and without leukopenia. No similarity was found between the HLA of the Yemenite Jews with leukopenia and that reported in black Africans with benign familial leukopenia. The suggestion of a genetic contribution from African blacks to Yemenite Jews is not supported by these results. The question remains to be answered whether the familial leukopenia in Yemenite Jews and black Africans is the result of a mutation.

Pleural effusion--presenting sign in multiple myeloma.

A patient with multiple myeloma in whom recurrent right pleural effusion was the presenting sign of the disease is reported. An IgA (k) monoclonal component was found in both the pleural effusion and the serum. The bone marrow specimen was interpreted as typical for multiple myeloma and the pleural fluid contained numerous plasma cells. Treatment with cyclophosphamide was followed by clinical improvement and the disappearance of the pleural effusion.