Hemophagocytic syndrome and T-cell lymphoma after kidney transplantation: a case report

Lymphoma in immunocompromised transplant patients is a feared cause of morbidity and mortality. Superimposed on the lymphoma and the transplantation immunosuppression is a rare condition: hemophagocytic syndrome (HS). HS is characterized by fever, hepatosplenomegaly and lymphadenopathy, skin rashes, jaundice, coagulopathy, and phagocytosis of blood elements with pancytopenia. Here we describe a rare but fatal case of a kidney transplant patient who developed T-cell lymphoma and HS, without evidence of EBV replication. A short review of the diagnosis, treatment, and prognosis of HS is given. Received: 4 March 1997 Received after revision: 6 June 1997 Accepted: 30 June 1997     

Relationship between dynamic balance and self-reported handicap in patients who have unilateral peripheral vestibular loss.

OBJECTIVE: The purpose of this study was to investigate whether Dizziness Handicap Inventory (DHI) score is related to postural performance as assessed by dynamic posturography. STUDY DESIGN: Retrospective study. SETTING: Outpatient in a tertiary referral center. PATIENTS: Ninety-two complete unilateral vestibular loss patients, categorized into 3 groups according to the postlesion stage: 1 to 2 months (n = 32; age, 47.6 +/- 10.7 yr), 4 to 7 months (n= 23; 47.1 +/- 8.37 yr), and 1 year and older (n = 37; 49.2 +/- 9.5 yr). MAIN OUTCOME MEASURES: Dizziness Handicap Inventory and dynamic balance measured with a seesaw platform moving either in the anterior-posterior or in the mediolateral direction. RESULTS: The mean DHI score was 25.8 +/- 18.7 and the range was 0 to 68. Dizziness Handicap Inventory scores did not differ significantly between the different unilateral vestibular loss groups studied. No difference was detected between the groups for the 3 subscores (emotional, functional, and physical), except that the older-than-1-year group had a significantly higher physical score than the 2 others. No correlation was found between DHI scores and postural indicators for either direction of the platform. However, patients unable to maintain balance when the seesaw platform moved in the mediolateral direction had significantly higher DHI scores than those who did not fall. CONCLUSION: Even if they are not directly related, we suggest that DHI and dynamic posturography are complementary approaches for appreciating the vestibular compensation process and are thus useful for postoperative counseling for vestibular loss patients.     

Agarose electrophoresis and inhibitor tests for isoamylase determination can give complementary clinical information

We report the presence of an extremely high proportion of "aged" amylase in the serum and cyst fluid of a patient with a pancreatic pseudocyst. A salivary amylase inhibitor test helped us to differentiate these "aged" pancreatic amylases from salivary fractions having a similar electrophoretic mobility.     

Autoimmune thyroiditis: a condition related to a decrease in T-suppressor cells

Monoclonal antibodies recognizing specific antigenic determinants of peripheral T-lymphocytes (OKT3PAN), helper T-cells (OKT4IND) and suppressor T-cells (OKT8SUP) were used to study the immune regulation in autoimmune thyroiditis. An indirect immunofluorescence microscopy method was employed to quantify the number of different subtypes. In addition, B-lymphocytes were also studied using a fluorescent surface Ig detection technique. Patients with autoimmune thyroiditis--independent of their clinical status--show a decrease in the number of suppressor T-lymphocytes. This finding, in agreement with other functional tests, indicates that the autoimmune phenomenon is linked to a decreased T-suppressor activity.     

Clonally related IgA- and IgE-secreting plasma cells in a myeloma patient

OBJECTIVES: The purpose of this work was to study the clonal relationship between the cells that secrete monoclonal proteins in an IgA/ IgE double multiple myeloma patient. Double monoclonal gammopathy is a rare condition in which two types of monoclonal proteins can be found in the serum and/or urine of patients with multiple myeloma or gammopathy of undetermined significance. The study of the relationship between the cells expressing the different monoclonal proteins may provide insight in the pathogenesis of these disorders. METHODS: The clonal relationship of the two tumoral plasma cell populations was examined by immunophenotyping and sequence analysis of the variable regions of the immunoglobulin heavy chain genes. Both immunoglobulin sequences were isolated from the bone marrow using a polymerase chain reaction (PCR)-based cloning strategy. Rare isotype-switch variants were detected by a myeloma-specific PCR in combination with different isotype-specific primers. An in vitro culture system, based on the activation of the CD40 molecule on the B cell, was used in order to isolate and expand myeloma-related B cells from peripheral blood that could possibly be regarded as myeloma precursor cells. RESULTS: The variable parts of the immunoglobulin heavy chains linked to either Calpha or Cepsilon were exactly the same, including the same somatic mutations. From the in vitro CD40 cultures B cells could be isolated that either expressed IgA or IgE with exactly the same variable immunoglobulin part as the myeloma clone. No pre-switched IgM myeloma-related B cells could be found. CONCLUSION: Both cell populations in this IgA/IgE myeloma patient shared a common clonal origin. No evidence for a pre-switched IgM precursor myeloma cell was found in this patient.     

Cystic fibrosis: an unusual cause of chronic pancreatitis

Chronic pancreatitis is most frequently associated with alcohol abuse. This should however not always automatically be accepted as the presumed cause. When the history is doubtful, uncommon etiologies must be considered as is illustrated by the present case. A 38 years old man was in the past 20 years treated for chronic pancreatitis ascribed to ethylisme although he always denied this. When the diagnosis was eventually questioned, new investigations showed slightly elevated sweat electrolyte concentrations and a delta F508/R117H genotype compatible with cystic fibrosis (CF). Demonstration of mild respiratory abnormalities, obstructive azoospermia and CF in his brother supported this diagnosis. Although rarely, pancreatitis typically develops in the kind of CF patients with milder genotypes and less severe symptoms. Systematic analysis for genetic mutations in patients with idiopathic chronic pancreatitis (ICP) revealed however that this mild form of CF is a less exceptional cause than thought. As CF patients increasingly survive into adulthood this disease should be considered as a possible etiology in the differential diagnosis of pancreatitis at all ages.     

Evaluation of the safety and efficacy of amrinone in chronic obstructive lung disease with cor pulmonale

In a pilot study the effect of a bolus dose of amrinone intravenously (IV), 0.5 or 1 mg/kg body weight, in 10 patients with chronic obstructive lung disease and cor pulmonale was evaluated. We found that the higher dose of 1.0 mg/kg IV significantly (P less than 0.05) decreased the mean pulmonary artery pressure and pulmonary wedge pressure without significant changes in cardiac output, in systemic blood pressure or in arterial blood gas values. No adverse effects were recorded in any of the patients.