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[Rebound hyperplasia of the thymus with secondary intrathymic bleeding. Rare differential diagnosis of acute chest pain].

HISTORY AND ADMISSION FINDINGS: A 41-year-old man was admitted, in reduced general condition, having for several hours experienced acute left chest pain and dyspnoea. On examination there was dullness on percussion over the base of the left lung with decreased breath sounds. At the age of 28 years he had been placed on peritoneal dialysis for renal failure due to congenital kidney aplasia. A year later he had been given a renal transplant, which had to be removed a year before admission because of transplant glomerulopathy. He had been on peritoneal dialysis since then. INVESTIGATIONS: Haemoglobin concentration, erythrocyte count and haematocrit gradually fell. A chest X-ray revealed a mediastinum widened to the left. Computed tomography demonstrated a blood-containing mediastinal tumour and a haemothorax. DIAGNOSIS, TREATMENT AND COURSE: A haemorrhagic mediastinal tumour was excised at thoracotomy. Histology of the specimen showed thymus hyperplasia with extensive fresh bleeding in the thymus. The postoperative course was without complication and the patient's exercise capacity returned to normal. CONCLUSION: Thymus hyperplasia with life-threatening spontaneous haemorrhage can occur after termination of immunosuppression following transplant removal.

Adult↗

Intra- and extrathymic B cells in physiologic and pathologic conditions. Immunohistochemical study on normal thymus and lymphofollicular hyperplasia of the thymus.

Normal thymuses and thymuses with lymphofollicular hyperplasia have been examined immunohistologically using immunoenzymatic single and double labelling methods and a panel of monoclonal antibodies against B lymphocyte differentiation antigens (CD19-, CD20-, CD21-, CD22-, CD23- and CD37ag) and human immunoglobulins (IgM, IgD) for the presence and localisation of B lymphocytes and cells expressing B cell differentiation antigens. The numerous hyperplastic lymph follicles which occur in the pathological condition of lymphofollicular hyperplasia of the thymus were found to originate in the extrathymic compartment of the interlobular septal space. This area was found to be blown up by the growing lymph follicles with exactly the same cellular composition as their counterparts in the peripheral lymphatic tissue. Some of the B lymphocytes expressing the immunophenotype of follicular mantle zone lymphocytes which were detected in the thymic medulla probably infiltrated through discontinuities of the border between the perivascular space and the thymic medulla. Apart from this primarily extrathymic B cell compartment, B lymphocytes and cells expressing B cell antigens were found within the thymus medulla of normal control thymuses of different ages from fetal to adult life. These cells were detected as a small subpopulation in normal fetal, juvenile and adult thymuses. Morphologically they could be subdivided into small, round lymphoid cells accounting for less than 1% of medullary lymphoid cells, and into a larger variant, asteroidally shaped because of short cytoplasmic processes. These asteroid cells were even more infrequent than the lymphoid variant. Immunophenotype (CD19ag+, CD20ag+, CD22ag+, CD37ag+, IgM+, IgD+) and morphology of the first cell type led to the conclusion that the lymphoid cells were in fact B lymphocytes. They were scattered throughout the medulla of fetal and juvenile and adult thymuses alike. The second, the asteroid cell type, constantly expressed CD20ag and inconstantly IgM, CD22ag and CD37ag; furthermore, CD23ag was detected in a subset of the asteroid cells either restricted to the perinuclear zone or expressed in the entire cytoplasma and on the plasma membrane. The asteroid cells were located in the corticomedullary region of the fetal thymuses but were randomly distributed with a tendency to Hassall's corpuscles in juvenile and adult thymuses. They often formed rosettes with non-B lymphocytes.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗

Linkage of HLA to myasthenia gravis and genetic heterogeneity depending on anti-titin antibodies.

BACKGROUND: MG is an autoimmune disease of the neuromuscular junction. MG with thymus hyperplasia has been associated with, but not genetically linked to, the HLA-DR3 haplotype. OBJECTIVE: To re-evaluate the association of HLA with MG in 656 patients with generalized disease and to test linkage of HLA to MG with thymus hyperplasia. METHOD: Patients were genotyped for HLA-DRB1. Data analysis included case-control comparisons after subgrouping patients by thymus histopathology. The transmission of parental alleles to MG offspring with thymus hyperplasia was studied in simplex families using the transmission/disequilibrium test (TDT) as a test of linkage. RESULTS: MG with thymus hyperplasia was positively associated with DR3 (OR = 4.5, p = 1 x 10(-6)) and negatively associated with DR7 (OR = 0.28, p = 1 x 10(-6)), based on both case-control comparisons and TDT. No association was detected with thymomas. Conversely, patients who lacked thymus anomalies but expressed anti-titin antibodies (ATA) had an increase of DR7 (OR = 2.08, p = 4 x 10(-3)) and a decrease of DR3 (OR = 0.33, p = 9 x 10(-3)). CONCLUSIONS: The authors established linkage of HLA to MG and thymus hyperplasia, defining the MYAS1 locus. Moreover, DR3 and DR7, or closely linked genes, have opposing effects on MG phenotypes. Nonthymomatous patients with ATA may be a pathogenetically distinct subset of MG patients.

Adult↗

[Space occupying lesion in the anterior mediastinum in a patient with Basedow disease and previously diagnosed osteosarcoma].

HISTORY AND CLINICAL FINDINGS: A 24-year-old woman with osteosarcoma of the right thigh, diagnosed 12 years ago, complained at a follow-up examination of decreased exercise tolerance, increased nervous tension, heat intolerance, weight loss, hair loss and irregular stools. Examination revealed tachycardia (100/min), mild exophthalmus and a small goitre. INVESTIGATIONS: A decreased basal TSH level (0.002 mU/ml), raised peripheral thyroid hormone (fT4 6.7 ng/dl, total T3 7.8 micrograms/l) and a TSH receptor antibody titre of 33.4 U/l) were compatible with immune type Graves' disease. Radiology revealed an upper mediastinal space-occupying lesion which scintigraphically was separate from thyroid tissue. A metastasis of the osteosarcoma or thymus hyperplasia were considered the most likely cause. TREATMENT AND COURSE: The mediastinal lesion regressed under thyrostatic treatment with carbimazole (20 mg daily by mouth). But the clinical picture, localization and negative scintigraphy provided the diagnosis of transitory thymus hyperplasia in the course of Graves' disease. CONCLUSION: In immune type Graves' disease with a mediastinal space-occupying lesion, not only intrathoracic goitre but also thymus hyperplasia should be considered in the differential diagnosis.

Adult↗

Increased metabolic activity in the thymus gland studied with 18F-FDG PET: age dependency and frequency after chemotherapy.

UNLABELLED: This study was designed to evaluate the age dependency of 18F-FDG uptake in the thymus and the frequency of PET confirmation of thymus hyperplasia after chemotherapy in cancer patients. METHODS: Whole-body FDG PET recordings of 168 patients were retrospectively examined for a retrosternal lesion in the anterior mediastinum that was attributable to the thymus. The patients were assigned to the following four groups: children with malignant lesions before the first therapy (group Ia; n = 15; mean age +/- SD, 11.9 +/- 3.7 y), children with malignant disease after chemotherapy (group Ib; n = 12; mean age, 10.3 +/- 5.0 y), adults with histologically confirmed malignant lymphoma before the first therapy (group IIa; n = 37; mean age, 43.9 +/- 16.7 y), and adult lymphoma patients 3 wk to 4 mo after chemotherapy (group IIb; n = 104; mean age, 40.9 +/- 14.6 y). RESULTS: Increased FDG accumulation in the thymus was seen in 11 patients (73%) of group Ia and 9 patients (75%) of group Ib. Thymus hyperplasia was found in 5 patients (5%) of group IIb. The eldest of these 5 patients was 25 y old. No increased FDG accumulation in the thymus was observed in any of the group IIa patients. In cases of visible FDG uptake in the thymus, standardized uptake values did not exceed 4. CONCLUSION: FDG accumulation in the thymus is a common finding in children and can occasionally be observed in young adults after chemotherapy. Knowledge of the characteristics of a typical retrosternal lesion in conjunction with the clinical history allows avoidance of diagnostic uncertainty and unnecessary procedures.

Adolescent↗

[Thymus CT scan and thymoma associated antibodies in myasthenia gravis with thymoma].

OBJECTIVE: To investigate the clinical significance of the serum anti-titin, anti-ryanodine receptor (RyR) antibody level and thymus CT scan in the diagnosis of myasthenia gravis with thymoma. METHODS: Totally 32 patients with myasthenia gravis who had received thymectomy were included in the study. Abnormalities were shown under CT scan of thymus in all these patients. The relationships were studied among the pathological diagnosis, CT findings, and serum level of thymoma associated antibodies: anti-titin and anti-RyR antibodies. RESULTS: The pathological diagnosis of thymoma was made in 21 patients and thymus hyperplasia in 11 patients after operation. The sensitivity of CT scan in the diagnosis of thymomas was 90.5%, the specificity of serum thymoma associated antibodies in the diagnosis of thymoma was 100%. CONCLUSION: The thymoma-associated antibodies test is helpful in the differential diagnosis of thymomas and thymus hyperplasia; when combined with CT scan, it may achieve high sensitivity in the diagnosis of the thymoma.

Adult↗

[Hyperplasia of the thymus in Graves' disease. A case report].

Graves' disease is rarely associated with hypertrophy of the thymus which takes on a pseudotumor aspect. We observed a case in a 40-year-old woman who presented clinical and biological hyperthyroidism with anti-TSH receptor antibodies, favoring the diagnosis of Graves' disease which was confirmed by scintigraphy. The chest x-ray performed because of chest pain revealed enlargement of the mediastinum. The thoracic CT-scan without contrast injected showed a pseudotumor aspect of the thymus. Medical treatment with anti-thyroid drugs and beta blockers led to regression of the thymus mass. Knowledge of this type of association can avoid unnecessary thymus surgery. The clinical course is favorable irrespective of the type of anti-thyroid drug given.

Adult↗