Parotid gland involvement, the presenting sign of high grade non-Hodgkin lymphoma in two patients with Gaucher disease and sicca syndrome.

Increased risk of haematological malignancies has been described in Gaucher disease patients; however, high-grade lymphoma has been rarely observed. We report two patients with Gaucher disease and sicca syndrome diagnosed with aggressive lymphoma involving the parotid gland. A 29-year-old woman with Gaucher disease developed tumour of the left parotid gland. She reported chronic arthralgias, xerostomia and xerophthalmia. Parotid gland biopsy disclosed diffuse large B-cell lymphoma. No lymphadenopathy was found. Bone biopsy revealed focal lymphomatous infiltration consistent with stage IV disease. MACOP-B chemotherapy regimen (cyclophosphamide, adriamycin, methotrexate, bleomycin, vincristine, prednisone) resulted in complete remission for 15 years. A 76-year-old patient with Gaucher disease suffered from dry-mouth feeling. He developed a left parotid gland tumour. CT scan disclosed diffuse lymphadenopathy, pleural effusion and multiple lung nodules. A cervical lymph node biopsy revealed mantle cell lymphoma. Fine-needle aspiration of the parotid gland showed lymphoma cells. Immunochemotherapy with fludarabine, cyclophosphamide and rituximab resulted in complete remission. Accumulation of the glucocerebroside in Gaucher disease activates macrophages, inducing release of pro-inflammatory cytokines which may be involved in the pathogenesis of second malignancy. Patients with Gaucher disease bear an increased risk of haematological malignancies; however, aggressive lymphoma has been described only occasionally. In both our patients the presenting sign of lymphoma was tumour of the parotid gland. The patients suffered from sicca syndrome, which increases risk for developing lymphoma. The underlying Gaucher disease and sicca syndrome might be implicated as immunological triggers for lymphoma occurrence and its propensity for the parotid gland in these patients.

Epidemiology and ethnic aspects of B cell chronic lymphocytic leukemia in Israel.

Chronic lymphocytic leukemia (CLL) represents 30% of all leukemias in Caucasians. In East Europe and USA the disease incidence is high while in Asia and Africa CLL is rare. The present study deals with 302 cases of B cell CLL and related disorders; 207 patients originating from Europe and America (Ashkenazi Jews) and 95 descendants from Asia, The Mediterranean or Africa (Sephardic Jews). The patients were recruited during 1975-1996 in a single center covering the Hashfela region -- a Southern area of Israel with a current population of 430000 inhabitants. Incidence of the disease, clinical pattern, biological parameters, prognosis and outcome were investigated and compared in both ethnic groups. The results of this study show a high incidence of CLL in Israel. The mean annual age-adjusted incidence 4.3 per 100000 person-year is among the highest reported values. Our study confirms previous data on the prevalence of CLL in Ashkenazi compared to Sephardic Jews. The rise in CLL rate in the reviewed period occurred in both populations, mainly in the Sephardic group. The relative risk for Ashkenazies compared to Sephardics decreased from 6.0 in the 1975-1979 period to 2.4 in 1990-1996. A high rate of CLL was found in new immigrants from the former USSR with 26 cases de novo diagnosed and 11 prevalent cases not included in this series among approximately 60000 new immigrants in the ara over the last 8 years. No differences were found in clinical, laboratory and immunological parameters at the time of diagnosis in the two ethnic groups. The follow-up showed a similar pattern in the disease evolution. A preliminary study of immunoglobulin heavy chain rearrangement performed in 14 patients showed no significant differences in JH hybridization in the early stages of the disease, but more aberrations in advanced CLL in the Ashkenazi group. Our findings suggest that ethnic origin of the patients itself does not affect the biological and clinical behavior of this disease.

Human/mouse radiation chimera generated from PBMC of B chronic lymphocytic leukemia patients with autoimmune hemolytic anemia produce anti-human red cell antibodies.

Previous studies performed in our laboratory have shown that B-CLL cells are involved in the production of anti-red cell auto-antibodies, providing a possible mechanism for the autoimmune hemolytic anemia occurring during the course of B-CLL. In order to confirm this hypothesis, we attempted to transfer human B-CLL with AIHA to immunodeficient mice. Peripheral blood mononuclear cells (PBMC) from 11 B-CLL patients suffering from AIHA were transplanted into the peritoneal cavity of lethally irradiated Balb/c mice reconstituted with SCID bone marrow. Chimeric mice generated from PBMC of these patients (in stage III-IV of the disease) exhibited an engraftment profile with dominance of tumor cells and minuscule levels of T cells. Eighty-five percent of the chimeric mice generated from 10 out of the 11 B-CLL patients with Coombs'-positive AIHA, produced human Ig with anti-human red cell specificity as detected by indirect anti-globulin test. In addition, anti-red cell auto-antibodies were produced in 36% of chimeric mice generated from PBMC of Coombs'-negative B-CLL. In contrast, control experiments in which splenic cells from idiopathic AIHA or PBMC from normal donors were transplanted, failed to produce anti-RBC. This in vivo model further supports the relationship between the B cell expansion and the autoimmune hemolytic process.

Hydrochlorothiazide-amiloride causes excessive urinary zinc excretion.

Serum zinc levels and urinary zinc excretion were compared in 15 patients with essential hypertension taking chronically a combination of hydrochlorothiazide and amiloride as monotherapy, eight patients maintained with hydrochlorothiazide alone, and eight control subjects. Serum zinc values were statistically comparable in all three groups. However, urinary zinc excretion was abnormally elevated in the two patient groups. In the dosage used, amiloride did not have a zinc-sparing effect.

Post-cesarean section febrile morbidity. Antibiotic prophylaxis in low-risk patients.

A study was conducted to assess whether antibiotic prophylaxis in low-risk patients for post-cesarean febrile morbidity was beneficial and cost effective. In a randomized, prospective study, 167 patients received a single, 1-g dose of cefazolin before clamping of the cord, and 140 did not. In the group given prophylaxis the febrile morbidity and postoperative therapeutic antibiotic usage were significantly lower than in the group not given prophylaxis (9% vs. 17.9%, P = .035, and 6.5% vs. 20%, P < .001, respectively). We conclude that single-dose cefazolin prophylaxis is both beneficial and cost effective, even in patients considered to be at low risk of post-cesarean febrile morbidity. Since the value of antibiotic prophylaxis in high-risk patients is accepted, universal antibiotic prophylaxis in every cesarean section case is suggested.

Carcinocythemia. Report of two cases, one simulating a Burkitt lymphoma.

Carcinocythemia is a rare complication of metastatic carcinoma, characterized by the presence of carcinoma cells in the peripheral blood, which may mimic acute leukemia. Two cases are reported in which the patients developed carcinocythemia several years after being treated for carcinoma of the breast. Cytologic examination of peripheral blood smears in both cases showed the presence of numerous large abnormal cells; in one case the cells simulated those of a Burkitt lymphoma. Cytochemical and/or immunologic marker studies ruled out a hematopoietic origin of the malignant cells in both cases and confirmed a diagnosis of carcinocythemia. The rapidly fatal outcome observed in these two cases was in accordance with the poor prognosis usually encountered with this rare phenomenon.

Association of the Lewis blood-group phenotype with infertility in women.

OBJECTIVE: To evaluate the distribution of Lewis blood group phenotype and secretor status among women treated for infertility. SETTING: In vitro fertilization unit of a university hospital. PATIENTS: Forty-seven consecutive infertile women with mechanical (n = 31) or unexplained (n = 16) infertility scheduled for IVF-ET. The control group was formed of 47 fertile women from our database and additional new women matched for age. MAIN OUTCOME MEASURES: Determination of ABO and Lewis blood group phenotypes. RESULTS: Of the 47 subfertile women, 12 had blood type A (25.5%), 10 type B (21.3%), 4 type AB (8.5%), and 21 type O (44.7%); 38 had Le (a-b+) (80.9%), 4 had Le (a+b-) (8.5%), and 5 had Le (a-b-) (10.6%). Of the 47 controls, 17 had type A (36.2%), 12 type B (25.5%), 4 type AB (8.5%), 14 type O (29.8%); 26 had Le (a-b+) (55.3%), 11 had Le (a+b-) (23.4%), and 10 had Le (a-b-) (21.3%). The difference in the proportions of the A, B, AB, and O phenotypes was not statistically significant. The proportion of combined recessive and nonsecretor phenotypes Le (a+/-b-) was significantly lower in subfertile women (9/47) as compared with fertile controls (21/47) (P = 0.014). The difference in the proportions of the Lewis blood group phenotypes between the unexplained and the mechanical infertility groups was not statistically significant. CONCLUSIONS: Subfertile women have an increased frequency of the Le (a-b+) blood group phenotype. Our hypothesis is that the presence of exposed fucosylated determinants such as Le(b) on the surface of endometrial cells may interfere with implantation.

Blood transfusion reactions in elderly patients hospitalized in a multilevel geriatric hospital.

Background/Objectives. Blood transfusion is a critical issue for patients with chronic diseases such as heart failure, chronic kidney disease, and malignancy. However, side effects are not rare. The purpose of the study is to evaluate the frequency of adverse blood transfusion reactions in hospitalized elderly patients during a one-year period. Design/Setting/Participants. Blood transfusion reactions such as fever, chills, dyspnea, and others following blood transfusions in hospitalized geriatric patients during one-year period were examined. Results. 382 blood units (242 patients) were administered during the study period. In 40 (11%) cases, blood transfusion reactions occurred. Fever was the most common reaction in 29 cases (72%), four (10%) had shortness of breath, and 3 (8%) had vomiting and chills each. There were no lethal cases in the 24-hour period following blood transfusions. Conclusion. A relatively low rate of adverse blood transfusion reactions occurred in our geriatric patients. We may speculate that this is related to underreporting of minor symptoms due to the high percentage of demented patients in this population.

Smoking and choroidal thickness in patients over 65 with early-atrophic age-related macular degeneration and normals.

OBJECTIVE: To compare macular choroidal thickness between cigarette smokers, those with a history of smoking, and nonsmokers in patients over 65 years of age with early-atrophic age-related macular degeneration (AMD) and normals. METHODS: Prospective, consecutive, observational case series. Enhanced depth imaging spectral domain optical coherence tomography 12-line radial scans were performed and choroidal thickness manually quantified at 84 points in the central 3 mm of the macula. Data of normals, soft drusen alone, and soft drusen with additional features of early AMD were compared. A multivariate analysis of variance (MANOVA) model, controlling for age, was constructed to evaluate the effect of smoking history and AMD features on choroidal thickness. RESULTS: A history of smoking was significantly associated with a thinner choroid across all patients via logistic regression (P=0.004; O.R.=12.4). Mean macular choroidal thickness was thinner for smokers (148±63 µm) than for nonsmokers (181±65 µm) among all diagnosis categories (P=0.003). Subgroup analysis of patients with AMD features revealed a similar decreased choroidal thickness in smokers (121±41 µm) compared with nonsmokers (146±46 µm, P=0.006). Bivariate analysis revealed an association between increased pack-years of smoking and a thin choroid across all patients (P<0.001) and among patients with features of early AMD (P<0.001). Both the presence of features of macular degeneration (P<0.001) and a history of smoking (P=0.024) were associated with decreased choroidal thickness in a MANOVA model. CONCLUSION: Chronic cigarette smoke exposure may be associated with decreased choroidal thickness. There may be an anatomic sequelae to chronic tobacco smoke exposure that underlies previously reported AMD risk.

Direct antiglobulin test reactive with complement only in warm type autoimmune hemolytic anemia.

Direct antiglobulin test (DAT) with only complement detected on red blood cells is a rare laboratory finding, and its significance in the setting of warm autoimmune hemolytic anemia (AIHA) is controversial. During 2 years (2003-2004) 277 patients with positive DAT were recorded in the blood bank registries, 17 of them had DAT reactive with C3 alone with no cold agglutinin or other nonimmune causes for hemolysis diagnosed. Red cell eluate disclosed small amounts of IgG in two patients. In nine patients no signs of clinical hemolysis were found, however, all these patients had underlying conditions that are known to be associated with red cells autoantibodies (autoimmune disorder or malignancy). Eight patients developed AIHA, seven of them with severe hemolysis. Three patients had idiopathic AIHA, and the others have been diagnosed with infectious, lymphoproliferative and autoimmune disorders. In two patients with acute infection the hemolytic process spontaneously resolved, three responded to corticosteroid therapy, while three patients were refractory to two lines of drug therapy and underwent splenectomy. Reticulocytopenia was found in four patients. Our results emphasize that AIHA with DAT reactive with complement alone is a rare disorder and might be accompanied by severe, refractory to conventional treatment and life-threatening hemolysis.

The ABO, Lewis or P blood group phenotypes are not associated with recurrent pelvic inflammatory disease.

An assumption that ABO, Lewis, or P blood group phenotypes are associated with recurrent pelvic inflammatory disease (PID) in a similar way as with recurrent urinary tract infection has been tried to establish. Of 20 patients with PID 9 (45%) had blood type A, 6 (30%) type B, 1 (5%) type AB and 4 (20%) type O; 14 (70%) had Le(a-b+), 5 (25%) had Le(a+b-), and 1 (5%) had Le(a-b-). Of the 20 controls 10 (50%) had blood type A, 3 (15%) type B, 1 (5%) type AB and 6 (30%) type O; 12 (60%) had Le(a-b+), 4 (20%) had Le(a+b-), and 4 (20%) had Le(a-b-). The difference in the proportions of the A, B, AB, and O phenotypes as well as the proportion of combined recessive and nonsecretor phenotype Le(a+/-b-) between patients with recurrent PID and controls were not statistically significant. The distribution was consistent with that in the general population. 2 of the patient group (10%) and 6 (30%) of the controls had positive blood type P1 (Fisher's exact probability = 0.0958). The distribution of P1 between the patients and controls was opposite to that in the general population. We could not demonstrate association of ABO, Lewis or P blood group phenotypes with recurrent PID.

Head and neck carcinoma in Fanconi's anaemia--report of a case and review of the literature.

Fanconi's anaemia (FA) is a rare autosomal recessive syndrome characterised by progressive lethal pancytopenia, skeletal abnormalities, hyperpigmentation and increased chromosomal aberrations. A high incidence of leukaemia and hepatocellular and squamous cell carcinomas (SCC) have been reported in FA patients. A rare case of SCC of the dorsum of the tongue in a FA patient is presented. A review of the reported cases of head and neck carcinoma in FA patients shows a different male:female ratio than previously reported, and a high incidence of carcinoma of the tongue.

Th polyagglutination with fatal outcome in a patient with massive intravascular hemolysis and perforated tumor of colon.

Polyagglutination is a rare disorder which has been associated with intravascular hemolysis (Levene et al.: Transfus Med Rev 2:176-185, 1988). In this condition cryptantigens exposed on the red blood cell membrane agglutinate with compatible sera and with lectins. A 76 year-old man with an acute abdomen due to a perforated tumor of the colon and severe fatal intravascular hemolysis is described. Th polyagglutination of the red blood cells was found.

An esoteric occupational hazard for lead poisoning.

A case of life threatening lead poisoning was diagnosed clinically in a Jewish scribe and verified by appropriate laboratory studies. The special ink used by the scribe was found to contain lead in appreciable amounts. Eleven more asymptomatic subjects, both scribes and manufacturers of the ink, were studied and five were found to have subclinical lead overload. Handling or production of this ink is a potential hazard for lead intoxication.