Waldenström's macroglobulinemia associated with AA amyloidosis.

It is widely accepted that amyloidosis in Waldenström's macroglobulinemia (WM) is exclusively due to amyloid light-chain deposition. However, only a small number of previous reports have actually characterized the type of amyloid in WM. We now report the third patient with WM and amyloid A protein (AA) amyloidosis. This patient developed malabsorption, nephrotic syndrome, and orthostatic hypotension. AA was immunohistochemically demonstrated in the rectal biopsy. In conjunction with previous examples of AA amyloidosis, the present report raises the possibility that AA amyloidosis may also occur in WM patients.

The incidence of pseudothrombocytopenia in automatic blood analyzers.

UNLABELLED: The aim of the present work was to undertake an assessment of the incidence of pseudothrombocytopenia (PTCP) in patients referred for evaluation of thrombocytopenia in an outpatient hematology clinic. METHODS: Prospective assessment of 60 consecutive cases with platelet count < 100 x 10(9)/l in a hematology clinic during a 2-year period. RESULTS: PTCP was the second most common cause for low platelet count, with an incidence of 17%. Platelet count of patients with PTCP at presentation was 42 +/- 22 x 10(9)/l, and when re-analyzed on fresh samples, 208 +/- 39 x 10(9)/l. The relatively high prevalence of pseudothrombocytopenia in our series was due to a lack of microscopic inspection of the blood smear in the primary care laboratories and considerable delay in sample processing. CONCLUSIONS: PTCP should be considered in the assessment of low platelet count. While decreasing the transfer time of blood specimens may decrease PTCP incidence, microscopic inspection of the blood smear may avoid erroneous diagnosis of thrombocytopenia.

Severe tumor lysis syndrome following splenic irradiation.

A 72-year-old man suffering from non-Hodgkin's lymphoma is described. After failing to respond to chlorambucil-prednisone regimen for three months, he was treated by splenic irradiation (total dose 500 cGY) in 10 consecutive daily doses. Two days after the last irradiation, the patient developed acute tumor lysis syndrome (TLS) with extreme blood levels of uric acid (33.7 mg/dl), phosphorus (13.7 mg/dl), potassium (6.08 mEq/l), and calcium (6.8 mg/dl). It should be remembered that the acute TLS, which usually occurs following chemotherapy, can also be radiation-induced.

Adult onset Still's disease associated with Epstein-Barr virus infection in a 66-year-old woman.

Adult onset Still's disease (AOSD) is a distinct clinical entity which affects predominantly young adults aged 16-35 years. Onset in elderly individuals is exceptional. Several reports have suggested a viral trigger in the pathogenesis of this disease. We describe a 66-year-old woman who fulfilled the proposed diagnostic criteria of AOSD and suffered concurrently from acute Epstein-Barr virus (EBV) infection.

Primary biliary cirrhosis and systemic lupus erythematosus. A rare association.

Although the co-existence between primary biliary cirrhosis (PBC) and one or more autoimmune diseases is very common, the association with systemic lupus erythematosus (SLE) is believed to be rare. We describe a 37-year-old woman with PBC who developed clinical and serological features of SLE 4 years later. The anti-mitochondrial antibody (AMA) titers fell to undetectable levels during the acute phase of the SLE. At the same time, high titers of antinuclear antibodies (ANA) were detected. Changes in opposite directions in the AMA and ANA titers were also seen during remission of the SLE.

Recombinant human erythropoietin in the treatment of multiple myeloma-associated anemia.

UNLABELLED: Multiple myeloma (MM) is commonly associated with anemia. Several causes have been implicated but inadequate erythropoietin (Epo) production appears to be important. This single-institute open-label, non-comparative clinical trial was undertaken in order to evaluate serum Epo levels in patients with MM and to study the efficacy and toxicity of recombinant human Epo (rHuEpo) in the treatment of MM-associated anemia. MM patients with a baseline hemoglobin (Hb) level of < 11 g/dl received rHuEpo 150 U/kg 3 times/week subcutaneously, with a possible dose increase to 300 U/kg if no response was observed after 4 weeks. The study was designed for 12 weeks, although some responders continued rHuEpo. The study endpoints were determined by an increase in Hb and a decrease in blood transfusion requirements (BTR). Seventeen patients were enrolled in the study. The median serum Epo level was 150 mU/ml (range 11-232). Four patients did not complete the study for reasons unrelated to rHuEpo, but to their underlying MM. Twelve patients (70.6%) responded with an increase in their Hb levels. One patient (5.9%) responded partially. The median Hb level rose from 9.4 g/dl (range 7.3-10.7) at study commencement to 12.5 g/dl (range 9.0-15.2). Six of the 11 patients who were transfusion dependent enjoyed a complete abolition of BTR. The response was also interpreted as an improved quality of life: 3 patients reported a decrease of 1 level in their WHO performance status (PS) score; in 8 patients, the PS declined by 2 grades and 1 patient enjoyed PS reduction by 4 scores. Six patients continue to receive rHuEpo up to 18 months, with a good response and a smaller maintenance dose. Four patients reported flu-like symptoms, 2 suffered from a local irritation and 1 experienced a transient controlled elevation of blood pressure. SUMMARY: (1) Pretreatment endogenous serum Epo levels were relatively low in all patients studied with MM-associated anemia; (2) rHuEpo was well tolerated in these patients; (3) rHuEpo was highly effective in the treatment of anemia in MM, and (4) the response to rHuEpo is characterized by an increase in Hb levels, a reduction in BTR and an improvement in the WHO PS score.

Recombinant human erythropoietin in the treatment of myelodysplastic syndromes--response patterns.

Different response patterns to recombinant human erythropoietin (rHuEpo) administration to anemic patients with myelodysplastic syndromes (MDS) are described. The biology of rHuEpo effect on erythropoiesis in patients with MDS has not been elucidated. However, until more biological information is obtained, it could be prudent to consider these response patterns as guidelines in the treatment of MDS. In the small but interesting series of nine patients with MDS only one responded to rHuEpo within the treatment period of eight weeks. Two additional patients continued the treatment on their own, and after 16 weeks a response was noted for the first time. A third patient was treated for only six weeks and a delayed response was recorded while off treatment for ten weeks. This response was also recorded 16 weeks from treatment initiation-as in the other two patients. A fourth patient with MDS developed transfusion related hemosiderosis and during iron chelation therapy the RBC transfusion rate dropped to a rate lower than the rate needed before the rHuEpo treatment. It is emphasized that in non responders, non-routine approaches should be considered.

Secondary myelodysplastic syndrome in multiple myeloma--a study of nine patients with an attempt to detect myeloma patients at risk.

Over a follow-up period of ten years, nine of our 100 patients with multiple myeloma (MM), developed myelodysplastic syndrome (MDS, preleukaemia). MDS occurred 19-156 (median 35) months from the diagnosis of MM. Six patients presented with pancytopenia and no patients had active MM at the time of MDS diagnosis. Three patients were defined as having refractory anaemia (RA) and six as refractory anaemia with excess blasts (RAEB) or RAEB in transformation (RAEBT), according to the FAB classification. The clinical course is characterized by increasing red blood cell and platelet transfusion requirements, recurrent infections and bleeding episodes. All patients, except for one, died within 3 to 8 (median 5) months from MDS diagnosis. The causes of death were sepsis or bleeding; three patients underwent leukaemic transformation. Thus, the clinical course of this small group of myeloma patients who developed secondary MDS (sMDS), was similar to other series of patients with sMDS. Serial bone marrow examinations suggest an initial hypercellular phase, followed by a rapidly evolving preterminal hypocellular marrow. In an attempt to detect MM patients at risk of developing sMDS, the epidemiological (including ethnic), clinical and laboratory data of the 9 MDS patients at the time of the MM presentation were reviewed and compared to the other MM patients. No significant differences were observed between the two groups in most parameters, except for two. All MDS patients were Ashkenazi Jews and no patients of Sepharadic origin developed MDS. Also, no IgA-myeloma patient developed MDS. If these findings are confirmed in a larger series, it may point to subgroups at risk which may require a different approach.(ABSTRACT TRUNCATED AT 250 WORDS)

[Initial treatment of urinary tract infection with augmentin].

Urinary tract infections have been treated empirically according to the development of bacterial resistance, and semisynthetic penicillins have been out of favor. Only 12% (4/34) of our patients treated with augmentin failed to respond, compared with 20% of those on a combination of gentamicin and ampicillin.

Uveitis, a presenting symptom of malignant histiocytosis.

A patient presenting initially with bilateral uveitis was admitted 6 weeks later to the Department of Internal Medicine "A" because of fever, chills, and pancytopenia. Physical examination, ultrasonography and CT scan revealed hepatosplenomegaly only, without lymphadenopathy. A bone marrow trephine biopsy showed areas with a dense infiltrate of abnormal cells, displaying many mitotic figures. Some of the cells resembled better differentiated histiocytes and contained hemosiderin pigment or phagocytosed erythrocytes. Immunohistological tests confirmed the diagnosis of malignant histiocytosis (MH). A survey of the literature revealed two other cases with uveitis and MH.

[Sarcoidosis presenting with subcutaneous nodules].

Sarcoidosis is an uncommon disease with many types of presentation. A 32-year-old woman is described in whom subcutaneous nodules were the presenting symptom. This presentation is vary rare; the differential diagnosis includes rheumatic fever, rheumatoid arthritis, systemic lupus erythematosus and fungal infections.

Effect of an iron supplement on body iron status and aerobic capacity of young training women.

Serum iron deficiency has a high incidence in female athletes. We investigated the effects of a daily oral iron supplement, (160 mg) administered during an intensive 7-week physical training programme, on body iron status, and the maximal aerobic capacity (VO2max) of 13 women (group A) compared to 15 who took a placebo (group B). The subjects were 19 years old. Blood samples were obtained before training began and on days 1, 7, 21 and 42 of training. They were analysed for packed cell volume (PVC) and for haemoglobin (Hb), 2,3-diphosphoglycerate (2,3-DPG), haptoglobin, iron and ferritin concentrations. The VO2max was measured on days 0, 21 and 42 of training. Following 21 days of training Hb, PCV and ferritin were significantly higher (P less than or equal to 0.01) in group A compared to group B. Over the training period Hb rose by 9.3% and 2.4% in groups A and B, respectively. At the end of training 66% of group B exhibited ferritin concentrations below 10 ng.ml-1, while none of group A had such low values. Mean VO2max of group A had increased by 7.5% following 21 days of training (P less than or equal to 0.01) and by 15.3% after 42 days. No appreciable increase in VO2max had occurred in group B by day 21 (significantly lower than VO2max of group A; P less than or equal to 0.05), however by day 42 it had increased by 14.3% (P less than or equal to 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Hypouricemia due to familial isolated renal tubular uricosuria. Evaluation with the combined pyrazinamide-probenecid test.

A Jewish Iraqi woman with familial isolated renal tubular uricosuria, urate clearance of 60.5 +/- 5.7 ml/min and hypouricemia of 1.0 +/- 0.2 mg/dl is described. The combined pyrazinamide-probenecid test suggested a presecretory defect in urate reabsorption. Four offspring were moderately affected. This family represents the sixth Jewish Iraqi family with familial isolated presecretory uricosuria, and emphasizes the marked prevalence of this disease among the Iraqi Jewish population. Since the inheritance of the presecretory defect is autosomal recessive, we suggest that this family is an example of pseudodominant transmission. The combined pyrazinamide-probenecid test may cause a reduction in glomerular filtration rate and filtered load of urate and thereby affect urinary excretion rate of urate in patients with urate wasting.

Non-random chromosomal aberrations in a complex leukaemic clone of a Bloom's syndrome patient.

Bloom's syndrome is one of the congenital disorders known to have increased frequency of acute leukaemia. The complex cytogenetic findings in the leukaemic cells of a 39-year-old male with Bloom's syndrome are described. These included a translocation t(7;17), missing 7q and 17p, a reciprocal translocation t(4;22); del 3q, del 8q22, del 20q, missing 12 and missing Y. In the same patient a missing Y had been noted 10 years previously in 15% of his peripheral blood lymphocytes.

Dipyridamole-induced epistaxis.

The case history of a patient treated with dipyridamole who developed severe epistaxis, accompanied by alteration of the platelet aggregation tests, is presented. The bleeding did not respond to local therapy and stopped only after discontinuation of the drug and normalization of the platelet aggregation. Hemorrhage, especially in elderly patients, should be considered as a possible complication of dipyridamole administration.