A new ATTR Phe64Ile mutation with late-onset multiorgan involvement.

We describe a novel transthyretin mutation in which phenylalanine is replaced with isoleucine in exon 3 at codon 64: Phe64Ile. The mutation was found in an isolated patient and it was not possible to perform a family study. The phenotype included heart and peripheral nerve involvement associated with a possible gastrointestinal and renal involvement.

Smoking cessation at the workplace. Results of a randomised controlled intervention study. Worksite physicians from the AIREL group.

Objectives: To compare the effects of a worksite intervention by the occupational physician offering simple advice of smoking cessation with a more active strategy of advice including a "quit date" and extra support.

Population: Employees of an electrical and gas company seen at the annual visit by their occupational physicians. CRITERIA END POINTS: Smoking point prevalence defined as the percentage of smokers who were non-smokers at one year.

Modulation of osteoclast-activating factor activity of multiple myeloma bone marrow cells by different interleukin-1 inhibitors.

We have studied the effects of several interleukin-1 (IL-1) inhibitors--IL-1 receptor antagonist (IL-1ra), soluble IL-1 receptor (sIL-1R) types I and II, and neutralizing monoclonal antibody (mAb) specific for IL-1 receptor type I--on the osteoclast-activating factor (OAF) activity of recombinant IL-1beta and of culture supernatants of unfractionated bone marrow mononuclear cells from multiple myeloma (MM) patients. The latter activity sharply correlated with the IL-1 content of culture supernatants (r = 0.949; p < 0.

Circadian patterns in histamine concentrations and mast cell number in the rat thyroid gland.

We carried out a cross-sectional chronobiological investigation on blood histamine, thyroid histamine and thyroid mast cell number in Wistar rats. Daily, blood histamine varied from 0.38 +/- 0.

AL-amyloidosis in monoclonal gammopathies.

Sixty-two patients affected by MGUS underwent fat tissue aspirate examination for diagnosis of AL amyloidosis. Nine out of the 62 were found to be Congo red positive. MGUS had already been diagnosed for quite a long time in about 60% of these patients, while this prevalence decreased to 24% among the Congo red negative patients.

Daily rhythmic variations in histamine in human blood.

We examined whether or not normal subjects have rhythmic changes of blood histamine levels. Daily predictable variations are present with 3 maxima and 3 minima and acrophase at 09.13.

Recurrent oral condylomata acuminata and hairy leukoplakia: an early sign of myelodysplastic syndrome in an HIV-seronegative patient.

Oral hairy leukoplakia (OHL) has been observed in all risk groups seropositive for HIV infection. Recently, this lesion has also been described in HIV-seronegative patients with immunosuppression of iatrogenic origin. We report on a HIV-1 and HIV-2 seronegative, heterosexual man affected by refractory anemia with ringed sideroblasts (myelodysplastic syndrome), who developed recurrent oral condylomata acuminata and OHL as an early clinical manifestation.

Histamine uptake by human normal neutrophils in vitro.

The uptake of different concentrations of (2.5-3H) histamine by normal human neutrophils at 37.5 degrees C in a tris-albumin buffer was determined at 20, 60 and 90 min of incubation.

Production of interleukin-1 by bone marrow myeloma cells.

Plasma cells isolated from bone marrow (BM) aspirates of 12 patients with multiple myeloma (MM) and nine patients with monoclonal gammopathy of undetermined significance (MGUS) were analyzed for production of cytokines with bone-resorbing activity, such as interleukin-1 (IL-1), tumor necrosis factor (TNF), and lymphotoxin (LT). Culture supernatants of plasma cells from MM, but not from MGUS or normal donor, invariably contained high amounts of IL-1-beta and lower amounts of IL-1-alpha. With a single exception, TNF/LT biologic activity was not detected in the same supernatants.

Potential role of interleukin-1 as the trigger for diffuse intravascular coagulation in acute nonlymphoblastic leukemia.

Abnormalities of coagulation are common in patients with acute nonlymphoblastic leukemia, although the mechanisms involved are unclear, except in a few cases. To investigate the pathogenesis of this coagulopathy, suspensions of purified leukemic cells were prepared and tested for procoagulant activity. Neither the leukemic cells nor their supernatants directly accelerated the clotting of plasma.

Histamine uptake by leukocytes in vitro.

Human leukocytes at the concentration of 10(6) cells/ml were incubated for 1h at 37.5 degrees C in a tris-albumin buffer, containing 0, 6.25, 12.

[Glycosaminoglycans in the spleen of normal humans and in the spleen of subjects with chronic myeloid leukemia].

The average glycosaminoglycan content in control spleens, expressed as uronic acid, was 0.23 +/- 0.02 mg/g of dry wt; the average glycosaminoglycans content in spleens of CML patients, expressed as uronic acid, was 0.

[Cytochemical studies of bone marrow plasma cells in monoclonal gammapathies (author's transl)].

Three plasma cell enzymatic reactions--ATPase, acid phosphatase (AP), alpha-naphthyl acetate esterase (alpha-NAE)--were tested in a large number of bone marrow samples from patients with multiple myeloma (MM), benign monoclonal gammapathy (BMG)--idiopathic (iBMG) and secondary (sBMG)--, polyclonal hypergammaglobulinemia (PH), Waldenström's macroglobulinemia (WM) and from normoglobulinemic subjects (NS). The purpose of the present study was to evaluate the significance of these reactions in differential diagnosis of BMG and initial or atypical MM. For each reaction results were expressed as scores and normal limits were statistically established.

[The nature of crystals in plasma cells present in a case of apparently benign biclonal gammapathy].

A 77 years old white man presents, in his blood serum, a moderate gammapathy with 2 monoclonal peaks, IgAk and IgGlambda, and in his urine a little quantity of BJk, without any clinical or radiological manifestation. The sternal bone marrow showed 3-5% plasmacells, mostly containing many needlelike inclusions in the cytoplasm. These inclusions, red-violet at the May-Grünwald-Giemsa, were PAS and Sudan negative; positive at the Danielli reaction (for proteins).

Cytochemical detection of histamine in the human granulopoietic series of healthy subjects and of patients affected by chronic myeloid leukaemia. A spectrophotofluorimetric test for checking OPT-induced fluorescence in isolated cells.

Cellular histamine in blood and bone marrow has been identified histochemically using an o-phthaldialdehyde fluorescence reaction. The specificity of the reaction was tested by a spectrofluorometric analysis of cell extracts. In normal blood, the basophils emit a bright yellow fluorescence, whereas neutrophils, eosinophils and platelets react less consistently and when they do, they give off a less intense yellow or blue emission.

[Cytochemical demonstration and characterization of acid mucopolysaccharides in normal granulocytic series].

Metachromatic granulations are seen in myeloblasts, promyelocytes, myelocytes, metamyelocytes and young mature cells, after staining by all metachromatic dyes, except methylene blue. Furthermore by screening at various pHs granular metachromasia is detectable starting at pH 3.2 in 6% of case, at pH 4 in 78% of cases, at pH 4.