Two Cases of Autoimmune Neutropenia Complicated with Other Lineages of Autoimmune Cytopenia, Successfully Treated with Prednisolone.

Though adult-onset primary autoimmune pancytopenia (AIP) rarely follows a self-limited course, a standard treatment strategy has not yet been established. We herein report two cases, each involving primary autoimmune neutropenia complicated with autoimmune thrombocytopenia or Evans syndrome. They were refractory to granulocyte-colony stimulating factor, but all lineages of cytopenia promptly recovered with prednisolone (PSL). In case 1, PSL was tapered and discontinued six months after its initiation without AIP relapse. In case 2, PSL has been tapered for five months without relapse. To establish an optimal treatment strategy for AIP, it is necessary to accumulate more cases.

Vasculites pulmonares para o pneumologista: uma "cartilha introdutória" / Pulmonary vasculitis for pulmonologists: a 'introductory primer

Vasculites pulmonares são doenças raras, mas com grande possibilidade de produzir morbimortalidade nos indivíduos acometidos, não somente pela sua potencial gravidade multisistêmica, mas também por seu diagnóstico geralmente tardio. A identificação de padrões de doença que remetem a estes diagnósticos e a utilização de novos meios de investigação - incluindo ANCA (anticorpo antineutrofílico citoplasmático), reconhecendo suas vantagens e limitações - são importantes, se quisermos utilizar o moderno arsenal terapêutico da forma adequada e com sucesso. Atenção às resultantes clínicas é fundamental no acompanhamento de longo curso destes pacientes

Pulmonary vasculitis are rare illnesses, although possessing substantial morbi-mortality in affected individuals, not only due to its multisystemic nature, but also because of commonly delayed diagnosis. Disease pattern recognition that remind us of that group of diseases, and the use of modern investigation methods - including ANCA (anti-neutrophil cytoplasmic antibodies), with the knowledge of its pros and cons, are key features if the state of the art therapeutic modalities are to be properly used with success. Attention to clinical outcomes is fundamental in the long range management of these patients

AP-VAS 2012 case report: a case of lupus nephritis with predominant synchronous cellular crescent formation and myeloperoxidase-antineutrophil cytoplasmic antibody seropositivity.

Several cases with an overlap of antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (GN) and lupus nephritis (LN) features have been reported in recent years. However, the clinical and the pathologic features of this condition, including mode of development, histology, and response to treatment, are not fully understood. We report a 77-year-old woman who was diagnosed with Sjögren syndrome 15 years previously. The patient presented with acute worsening of renal function and was diagnosed with new-onset systemic lupus erythematosus. A renal biopsy specimen revealed proliferative LN with synchronous cellular crescents. She was also seropositive for myeloperoxidase-ANCA. Together with the positive staining for immunoglobulins and complement factors on immunofluorescence microscopy and scant subendothelial deposits by electron microscopy, we reached a diagnosis of ANCA-associated crescentic GN overlapping with LN. Although immunosuppressive treatment with methylprednisolone pulse therapy and intravenous cyclophosphamide followed by oral predonisolone was initiated, along with intermittent hemodialysis, these treatments did not induce remission of her GN. Therefore, she continued regular intermittent hemodialysis. However, she died because of candida pneumonia 4 months after admission. Generally, the glomeruli of patients with ANCA-associated GN exhibit different stages of crescents, namely cellular, fibrocellular, or fibrous. The histologically synchronous crescents in this case indicate that ANCA-associated GN overlapping with LN can progress more rapidly than that without LN. This overlapping type of GN may be resistant to conventional immunosuppressive therapies.

Two Cases of Autoimmune Neutropenia of Infancy / 임상소아혈액종양

Autoimmune neutropenia of infancy (AIN) is caused by increased peripheral destruction of neutrophils as a result of antibodies in patients' blood that are directed against their own neutrophils. Due to non-specific symptoms, benign clinical courses, and cumbersome diagnostic tests, AIN are commonly undetected. Antineutrophil antibody test for diagnosis of AIN has recently become available. Compared to its relatively lower absolute neutrophil count (ANC), the clinical course of AIN is mostly benign. Therefore, although treatment is not usually necessary for AIN, it is applicable in order to rule out other significant diseases, such as severe congenital neutropenia (SCN), which can be transformed to myelodysplastic syndrome or acute myelocytic leukemia. For this reason, several treatments can be used for neutropenia: granulocyte-colony stimulating factor (G-CSF) for SCN, trimethoprim and sulfamethoxazole (TMP-SMX) for prophylaxis. Here we report on two cases of AIN confirmed by indirect immunofluorescence test using flow cytometry.

Two Cases of Autoimmune Neutropenia of Infancy / 임상소아혈액종양

Autoimmune neutropenia of infancy (AIN) is caused by increased peripheral destruction of neutrophils as a result of antibodies in patients' blood that are directed against their own neutrophils. Due to non-specific symptoms, benign clinical courses, and cumbersome diagnostic tests, AIN are commonly undetected. Antineutrophil antibody test for diagnosis of AIN has recently become available. Compared to its relatively lower absolute neutrophil count (ANC), the clinical course of AIN is mostly benign. Therefore, although treatment is not usually necessary for AIN, it is applicable in order to rule out other significant diseases, such as severe congenital neutropenia (SCN), which can be transformed to myelodysplastic syndrome or acute myelocytic leukemia. For this reason, several treatments can be used for neutropenia: granulocyte-colony stimulating factor (G-CSF) for SCN, trimethoprim and sulfamethoxazole (TMP-SMX) for prophylaxis. Here we report on two cases of AIN confirmed by indirect immunofluorescence test using flow cytometry.

Antibiotic-induced severe neutropenia with multidrug-dependent antineutrophil antibodies developed in a child with Streptococcus pneumoniae infection.

Drug-induced neutropenia (DIN), particularly that in which antibiotic-dependent antineutrophil antibodies have been detected, is a rare disorder. We report the case of a child with pneumococcal pneumonia, who experienced severe neutropenia during various antibiotic treatments. We detected 4 kinds (cefotaxim, augmentin, vancomycin, and tobramycin) of antibiotic-dependent antineutrophil antibodies by using the mixed passive hemagglutination assay (MPHA) technique with this child.

Antibiotic-induced Severe Neutropenia with Multidrug-Dependent Antineutrophil Antibodies Developed in A Child with Streptococcus pneumoniae Infection

Drug-induced neutropenia (DIN), particularly that in which antibiotic-dependent antineutrophil antibodies have been detected, is a rare disorder. We report the case of a child with pneumococcal pneumonia, who experienced severe neutropenia during various antibiotic treatments. We detected 4 kinds (cefotaxim, augmentin, vancomycin, and tobramycin) of antibiotic-dependent antineutrophil antibodies by using the mixed passive hemagglutination assay (MPHA) technique with this child.