Immunoglobulin G4-related lymph node disease with an orbital mass mimicking Castleman disease:A case report

BACKGROUND Immunoglobulin(Ig)G4-associated diseases are a group of systemic diseases involving multiple organs and are also known as IgG4-associated sclerosing diseases.IgG4-associated lymphadenopathy occurring in the lymph nodes is characterized by a lack of specificity due to its clinicopathological characteristics and must be differentiated from a variety of lesions,such as Castleman disease,lymphatic follicular reactive hyperplasia,and lymphoma.CASE SUMMARY A 65-year-old male patient,with Guillain-Barre syndrome for 5 years,presented to our hospital complaining of bilateral orbital mass for 2 years.After hospitalization,the results of the patient’s laboratory tests showed that immunoglobulin subgroup IgG4 was 33.90 g/L and IgG was 30.30 g/L,but serum interleukin-6 was normal.The pathological morphology of orbital mass and cervical lymph node were consistent,which showed that a large number of plasma cells and eosinophils were observed in the lymphatic follicles,and the interstitial fibrous tissue was proliferative.Immunohistochemistry showed that CD20(B cells)(+),CD3(T cells)(+),CD38(+),IgG(+),IgG4 positive cells>100/high powered field,and IgG4/IgG>40%.Combined with clinical and immunohistochemical results,lymphadenopathy was consistent with Castleman disease-like IgG4-associated sclerosing disease.Prednisone acetate treatment was given at 40 mg/d.After 2 wk,the superficial lymph nodes and orbital masses shrank,and the IgG4 level decreased.As prednisone acetate was regularly used at a reduced dosage,no recurrence of the disease has been observed.CONCLUSION This case suggested that it is necessary to proceed cautiously in clinical practice with such patients,and immunoglobulin,complement,interleukin-6,C-reactive protein,and other examinations should be performed to confirm the diagnosis.

THE IMMUNOHISTOCHEMISTRY AND QUANTITATIVE MORPHOMETRIC STUDY OF MUCOSA ASSOCIATED LYMPHOID TISSUE(MALT)LYMPHOMA IN STOMACH

Objective: To invetigate the Immunohistochemistry characters and quantitative nmorphometric significance for the mucosa associated lymphoid tissue (MALT) lymphoma of stomach in 14 patients. Methods: The routine paraffin slides were cut, stained with H.E., and immunochemically by ABC method. The morphologic appearance of nuclei of lymphoma cells were measured with HPIAS-1000 color pathology picture analysis system. Results of the 14 cases, 9 was centrocyte like (CCL) cell lymphoma, 2 CCL with large cell lymphoma, 1 small no cleaved cell lymphoma, 1 large no cleaved cell lymphoma, 1 T immunoblastic malignant lymphoma. The morphologic measurement results showed that there were great significant differences (P<0.001) for the 15 items of morphology parameters between the nuclei of MALT lymphoma cells and those of normal control lymphocytes in stomach. There were great significance differences (P<0.001) or significance (P<0.05) for the most of the 15 items of morphologic parameters of nuclei among the 5 types of MALT lymphoma. Especially, that the values of area, circumference, equivalent diameter, area volume, circumference volume, long diameter, short diameter, practical area were increasing as the malignant degree of classification was rising, which reflect the increasing malignancy of the tumor. Conclusion: It was suggested that with the quantitative morphology measurement method, man could make accurate diagnosis for MALT lymphoma. It offered us a new method to make the diagnosis, so that it had significance. It might be also practicable with morphology measurement method to make the sub classification of MALT lymphoma.

Histopathologic findings of keratoconus corneas underwent penetrating keratoplasty according to topographic measurements and keratoconus severity

AIM： To investigate the histopathologic and morphological changes of the corneas with keratoconus（KC） undergoing penetrating keratoplasty（PKP） according to topographic findings and severity of KC.METHODS： The corneal tissue of 35 samples with KC was retrospectively evaluated with conventional light microscopy. Topographic and pachymetric parameters of keratoconus corneas by means of Pentacam such as mean keratometry（K） and central corneal thickness（CCT） were recorded. Severity of KC was graded according to Amsler-Krumeich classification. RESULTS： Epithelial thinning and breaks in Bowman＇s layer are the most common findings in keratoconus corneas（94.3% and 82.9% corneas, respectively）. The results revealed statistically significant higher mean K value and lower CCT in the keratoconus corneas that were affected by epithelial thinning, breaks in the Bowman＇s layer, folds in the Descemet＇s membrane, epithelial scars, breaks in Descemet＇s membrane, and stromal scars than those corneas without these findings（P〈0.05）. Moreover, those corneas with epithelial thinning, breaks in the Bowman＇s layer, folds in Descemet＇s membrane, epithelial scars, and stromal scars had significantly more severe disease than those corneas without these findings（P〈0.05）. The presence of the stromal and epithelial scars were associated with the higher KC severity, in which, respectively, 87.5% and 80.0% of the corneas with stromal and epithelial scars had stage 4 of the KC severity. CONCLUSION： It seems that there are some specific patterns in histologic changes of the keratoconus corneas. The presence of pathologic findings was correlatedwith thinner and steeper corneas. Epithelial or stromal scars were associated with the highest disease severity. The description of histopathologic findings of KC may help in elucidating the pathogenesis of the disease and help pathologist in differentiating KC from other corneal diseases.