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Summary Yersinia entercolitica endocarditis has rarely been described before. This is the first report of prosthetic valve Yersinia enterocolitis endocarditis, complicated by infected brain embolization. The patient, however, completely recovered after 6 weeks of combined therapy with ceftriaxone and gentamicin.

Leukocyte endothelial interactions are essential for a normal immune response. It is known that this response is influenced by stress and that the latter induces demargination. We examined the question of whether stress demargination results from a decreased state of leukocyte adhesiveness. Included were various volunteers and patients under different degrees of stress. 66 young athletes before beginning their daily exercises, 67 middle-aged healthy volunteers, 25 patients before ergometry for evaluation of chest pain, 75 patients who were referred to the emergency room with chest pain without ischemia/infarction, 78 patients with ischemia/infarction, 65 patients with minor trauma, 25 with a fracture and 12 with polytrauma. The leukocyte adhesiveness/aggregation (LAA) values were measured with a direct slide test. The respective LAA values were 7.4 +/- 4.7, 6.3 +/- 4.4, 5.8 +/- 3.6, 5.2 +/- 3.5, 10.8 +/- 8.5, 9.1 +/- 5.8, 12.2 +/- 6.6 and 19 +/- 12.6% of aggregated leukocytes. We conclude that an increase in aggregated white blood cells can be detected in the circulating pool during major stress. It is therefore suggested that stress demargination is not necessarily a result of diminished leukocyte adhesiveness.

A patient with endocarditis caused by Corynebacterium CDC Group A-4 is described. This is the first case in the literature of endocarditis caused by this bacteria, and is unique in that the patient was immune competent and the infection occurred on a native valve. This case illustrates that corynebacteria cannot be considered a contaminant and that the exact pathogen should be identified.

Summary The association between the CREST (calcinosis, Raynaud phenomena, oesophageal hypomotility, sclerodactyly and telangiectasia, variant of systemic sclerosis and chronic lymphocytic leukaemia is described in three patients. The present description raises the possibility that the association of chronic lymphocytic leukaemia and systemic sclerosis is non-random.

Cryoglobulinemia with peripheral cryoglobulinemic neuropathy is reported in a patient with Gaucher's disease. To the best of our knowledge, a similar relationship has not been previously described. The question whether the cryoglobulinemic neuropathy in this patient is related to Gaucher's disease, or is a mere coincidence, is discussed.

After 3 years of treatment with azathioprine and prednisone, immunoblastic lymphoma appeared in a patient with systemic lupus erythematosus. An increased incidence of immunological aberrations and malignancies was found in her family and their relation to the pathogenesis of this patient's diseases is discussed.

A patient with chronic myelocytic leukemia in whom a clinical and hematological remission occurred after a single course of busulfan is described. The remission lasted for a period of 13 years and during that lapse of time, she gave birth to a healthy child. Afterwards, acute myeloblastic leukemia was diagnosed. Following treatment with rubidomycin and arabinoside C another remission was achieved, lasting already for 18 months.

The association between aggregates of leucocytes in blood drawn from patients with various inflammatory conditions and the serum concentration of C-reactive protein (CRP) was examined: serum concentration of CRP might contribute to the development of cellular aggregations. A total of 213 patients with various inflammatory or necrotic conditions were examined (including 31 women with normal pregnancy and 59 controls). A significant correlation between the degree of leucocyte aggregation and CRP concentration was noted in patients with bacterial infections and in a group of patients with various inflammatory conditions. In contrast, there was no correlation between the extent of leucocyte aggregation and CRP concentrations in patients with viral infections, malignancies, or pregnancy. The presence or absence of aggregated leucocytes can help in differentiating between the respective bacterial or viral infections. The serum concentrations of CRP were increased in both types of infection, although when a quantitative CRP assay was used, considerably higher concentrations were detected in bacterial diseases.

The coexistence of cryoglobulinemia and C4 deficiency was found in one family. Twelve subjects were examined, most of them being asymptomatic. Cryoglobulins were found in 9 members and its type was defined in 6 of them, 3 having mixed IgM-IgG and 3 having monoclonal IgM cryoglobulins. The concentration of the third component of the complement system was normal in 10 subjects, while the concentration of the fourth one was found to be below the lower limit of normal in 9 subjects. Only C4 deficiency--and not cryoglobulinemia--was HLA-linked. There was no evidence of HBs antigenemia. The results of this study are discussed in the light of the pertinent literature.

A heterozygous state for C4 deficiency was found in 14 members of a family with familial SLE. The HLA analysis in this family permitted us to re-establish the linkage assumed to exist between the gene (or genes) controlling the synthesis of the fourth component of the complement and the major histocompatibility complex. In addition, it was found that the complement deficiency is not necessarily associated with any serological or clinical abnormality. The heterozygous state for C4 deficiency can be added to the various serological abnormalities described in asymptomatic members of families with familial SLE. It is as yet unknown whether this deficiency may predispose to the subsequent development of SLE.

Six out of 8 healthy first-degree relatives of a patient with systemic lupus erythematosus (SLE) had very high serum levels of a common anti-DNA idiotype (16/6). In this family an additional sister developed SLE, and all members were heterozygous for C4 deficiency. Measurements of common autoantibody idiotypes may contribute to the understanding of the genetics of autoimmune diseases. They might be found useful in detecting healthy subjects prone to the development of an overt clinical state.

To the Editor.— Serious side effects, including aseptic meningitis, were noted in several systemic lupus erythematosus (SLE) patients treated with ibuprofen (239:1062, 1978). 1-3 We presently describe a specific cell-mediated immunity (CMI) to ibuprofen in SLE. The study group consisted of 11 women with SLE—two treated with ibuprofen, while the others never received this agent. Sixteen patients with active rheumatoid arthritis (RA) (five of them treated with ibuprofen) as well as ten healthy persons served as control subjects. In seven of the 11 SLE patients, the disease was active at the time of examination, as evidenced by high titers of antinuclear factor, anti-DNA antibodies, and low titers of C3 and C4 complement components. All but one patient were treated with antiinflammatory drugs, including aspirin, indomethacin, phenylbutazone, or corticosteroids. In the SLE patients treated with ibuprofen, 800 to 1,200 mg/day, maculopapular skin rashes developed on the trunk and extremities. Fever occurred

Benign neutropenia is often found among healthy Yemenite Jews. An assessment was made of the magnitude of labor-induced leukocytosis in 44 Yemenite Jewish women, by comparing their hematological values during labor with those of 27 non-Yemenite women. A statistically significant difference was found in the absolute counts of the white blood cells (WBC) during delivery between the two groups, the values being lower among Yemenite Jews [10,291 +/- 422] vs. [11,759 +/- 630] X 10(9)/1) and similar findings were detected among their infants. The low WBC counts in the Yemenite group at parturition were also associated with significantly lower plasma cortisol levels (Yemenite group: 33.7 +/- 3.5 micrograms/dl; controls: 49.3 X 2.9 micrograms/dl). A correlation was found between serum cortisol levels and the magnitude of leukocytosis in both groups of women and their infants. These results suggest that a low basal corticosteroid output may contribute to the low white blood cell counts detected in some ethnic groups like the Yemenite Jews.

To the Editor.— Specific infiltrations in the mouth, while relatively frequent in the acute forms of leukemias, are rare in chronic lymphatic leukemia (CLL). 1,2 We presently report a CLL case in which a huge leukemic infiltration in the hard palate was diagnosed. Report of a Case.— A 77-year-old housewife, known to have CLL since 1972, was treated with chlorambucil and cyclophosphamide, monitored according to her blood counts. Her condition was satisfactory till June 1977, when she noted a painful swelling in her hard palate where a necrotic ulcer, 3×2 cm with regular and smooth border, was seen. A biopsy specimen from the latter showed massive infiltration with small lymphocytes, consistent with the diagnosis of CLL. A local cobalt irradiation (400 rads) completely healed this lesion. Comment.— Oral manifestations such as gingival hypertrophy, hemorrhages, ulcerations, necroses, and infections, while common in the acute forms of leukemia, are rare in CLL

A patient with acute myelogenous leukemia developed severe hypophosphatemia manifesting by extreme weakness, confusion, loss of sphincter control, nuchal rigidity, hyperesthesia, hemolysis, congestive heart failure and liver dysfunction. The possible causes for this condition were starvation, parenteral glucose and saline administration, sepsis, hypokalemia and treatment with acetazolamide. A dramatic improvement was noted following phosphate administration.

Leukocytosis (WBC counts 10,000/mm3) was detected in 77 out of 252 patients (30%) with ten different types of nonhematological malignancy (NHM) at the time of diagnosis. A full search including serological and bacteriological screening was performed to exclude other possible causes of leukocytosis. Among the different tumors, carcinomas of the lung and colorectum were the most prevalently associated with leukocytosis. Absolute monocytosis was found in 25% of the patients and absolute eosinophilia in only 4.8%. The leukocytosis was attributed mainly to an increase in the mature polymorphonuclears, suggesting a release mechanism of WBC from storage pools by factors secreted or induced by the tumor. Neither the age nor the sex of the patients affected the incidence or magnitude of leukocytosis. However, the presence of metastases was associated with a significantly higher incidence of leukocytosis (p less than 0.05). The associated leukocytosis may be regarded as a poor prognostic sign, and was associated with a significantly (p less than 0.007) shorter survival time. In contrast, absolute lymphocytosis may have a positive effect on the survival time (p = 0.01). Tumor-associated leukocytosis may be an additional tumor-associated marker, of value in assessing and monitoring patients with NHMs.

A 25 year old woman suffering from recurrent attacks of hypoglycaemia underwent a laparotomy for suspected insulinoma. No tumour was found, but histology showed islet cell hyperplasia and nesidioblastosis. Although these changes have been reported as a cause of hypoglycaemia in infants, they are only rarely the cause of hypoglycaemia in adults; in the present case they were found to be the result of covert sulphonylurea administration. The fact that sulphonylureas can cause nesidioblastosis is not well recognised by either physicians or pathologists and it should be considered before "cryptogenic nesidioblastosis" is deemed the cause of hypoglycaemia in adults.

Glycosphingolipids were isolated from the leukocytes of 10 patients with Type I, chronic nonneuronopathic (adult) Gaucher's disease and 12 normal subjects, by silicic acid column chromatography and thin-layer chromatography. Quantitation of the individual glycosyl ceramides was achieved by the determination of hexose and sphingosine content, using colorimetric and fluorometric procedures. Lactosyl ceramide, which is the main glycolipid in leukocytes of normal subjects, was significantly increased in the leukocytes of patients with Gaucher's disease. On the other hand, the amount of glycosyl ceramide, which is the main glycolipid accumulating in the reticuloendothelial cells of patients with Gaucher's disease, was similar in Gaucher and in control leukocytes.

Erythrocytes of patients with polycythemia vera exhibited increased activity of oxidative pentose shunt, accelerated generation of phosphoribosylpyrophosphate, increased incorporation of 14C-adenine into nucleotides, and increased activity and thermostability of adenine-phosphoribosyltransferase. These abnormalities are attributed to age dependency of the pathways concerned and presence in polycythemia vera of an erythrocyte population younger than normal.

The review of the pertinent literature disclosed 36 reports of familial multiple myeloma, described mostly in siblings, to which the authors add one more family. These patients did not differ significantly from those with non-familial myeloma with regard to sex, age, distribution of monoclonal proteins, clinical and laboratory data, and the course and prognosis of the disease. An increased incidence of immunoglobulin abnormalities was observed in healthy relatives of patients affected with familial myeloma. In most cases the time interval of the diagnosis of myeloma in a family member of a known patient was under 4 years. These observations, in conjunction with reports of myeloma occurring in clusters in a community and the appearance of myeloma in spouses raise the possibility of an environmental factor (virus?) which may contribute to the pathogenesis of myeloma in genetically predisposed individuals. Multiple myeloma should be added to the list of neoplastic diseases in which the family history is relevant and in which genetic and possibly environmental factors may be pathogenetically involved.

A 62-year-old woman who developed systemic lupus erythematosus 6 years following chemotherapy for malignant lymphoma is reported.

Two prolonged remissions were achieved in a patient with chronic myeloid leukemia by two short courses of busulfan treatment. The first remission lasted for 7 years; the second one lasts already 14 years. In the interval periods no treatment was administered.

A case of Gaucher’s disease is presented, in which prolonged partial thromboplastin time was found. The possibility of circulating sphingolipids responsible for this abnormality is raised.