A rare manifestation of IgG4-related disease and secondary hypereosinophilic syndrome: A case report.

We report a case of IgG4-related disease (IgG4-RD) with marked eosinophilia. A 79-year-old woman was admitted due to diarrhoea and weight loss. Cervical lymphadenopathy, bilateral submandibular glands swelling, anaemia (Hb8.5 g/dl), hypereosinophilia (9750/µl), elevated serum creatinine (1.57 mg/dl), pancreatic amylase (191 IU/l), and IgG4 (3380 mg/dl) were found. Diffusion-weighted image on magnetic resonance imaging showed high-intensity signals inside both the pancreas and the kidneys. The echogram of submandibular glands revealed cobblestone pattern. Kidney biopsy revealed acute tubulointerstitial nephritis. Biopsies of lip, gastrointestinal tract, and bone marrow showed infiltration of lymphoplasmacytic cells and IgG4-positive plasma cells (30-67/HPF). Gastrointestinal and bone marrow biopsies also showed eosinophilic infiltration. Adrenal insufficiency, rheumatic disease, tuberculosis, parasite infection, drug-induced eosinophilia, and eosinophilic leukaemia were all ruled out. We started treatment with 40 mg of prednisolone (PSL) and her general condition rapidly improved. The eosinophil count, serum IgG4, and serum creatinine decreased. We gradually tapered PSL and maintained 5 mg/day. During the 5 years of treatment, she had no recurrence of the symptom. According to the 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-RD, eosinophils >3000/µl is one of the exclusion criteria. If we comply with this criterion, the diagnosis of IgG4-RD should be avoided. However, our case fit the diagnostic criteria of type I autoimmune pancreatitis, IgG4-related sialadenitis, and global diagnosis of IgG4-RD. We finally diagnosed our case as IgG4-RD with secondary hypereosinophilic syndrome. This case suggests that IgG4-RD with eosinophils >3000/µl does exist in the real world.

Toxocariasis en adulto manifestada como síndrome hipereosinofílico con compromiso neurológico predominante: caso clínico / Toxocariasis manifesting as an hypereosinophilic syndrome with predominant neurological involvement: report of adult case

Hypereosinophilic syndrome is characterized by persistent hypereosinophilia and signs or symptoms due to organ involvement, specially nervous system, heart and skin. It can be primary or secondary to allergies, parasites or cancer. Toxocariasis is an uncommon parasitic disease in adults. There is a variant, called visceral larva migrans, that can involve different organs, and among those, the central nervous system. We report a 61 years old male, with a cerebrovascular disease. There were focalizing symptoms, the CAT scan showed multiple ischemic lesions and a peripheral eosinophilia of 12152 cells/mm3 was present. Anti toxocara IgG antibody titers were 1/1000. The patient was treated with albendazole for 14 days. After a 2 years follow up the patients is in good conditions and, for the first time, his eosinophil count is within normal limits

Considerações sobre a hipereosinofilia na paracoccidioidomicose infantil: apresentação de um novo caso / Considerations about hypereosinophilia in child: new case presentation

É apresentado o caso de um menino de seis anos de idade, natural e procedente de Paracambi (RJ), cuja doença durava dois meses, com manifestações respiratórias e gerais, como febre, sudorese noturna, anorexia e perda de peso. Posteriormente, surgiram dores abdominais, adenomegalias superficiais e massa abdominal palpável. Os hemogramas efetuados revelaram notáveis hipereosinofilias, o primeiro deles mostrando aproximadamente 24.000 eosinófilos por mm³. O diagnóstico de paracoccidioidomicose foi feito por biópsia de gânglio, tendo o esquema de anfotericina B e sulfametoxazol + trimetoprima conseguido excelente resposta clínica. São discutidas as eosinofilias e hipereosinofilias na PCM, conferindo-se maior ênfase aquelas observadas na infância e fazendo-se considerações sobre seu diagnóstico diferencial

Larva migrante visceral (toxocariasis humana) causa de hipereosinofilia y granulomas viscerales en el adulto / Visceral larva migrans (human toxocariasis) cause of hypereosinophilia and visceral granulomas in the adults

A 24-year -old woman 2-3 months after a normal parturation presented geophagy. Due to hypermenorrhea she consulted a gynecologist and in a hemogram a 57 percent (6, 893 x mm3) hypereosinophilia was detected. A chest TAC showed bilateral pulmonary nodules. The following tests resulted positive: ELISA IgG for toxocariasis 1: 1000, isohemagglutinins anti A 1:2048 and anti B 1:512. The patient was treated with albendazole and prednisone during 10 days. One month after treatment eosinophilia decreased to 2.590 x mm3 and ELISA IgG for toxocariasis descended to 1:128. Different aspect of human toxocariasis are commented. When hypereosinophia is observed in adult patients, toxocariasis must be checked