Uma rara causa de dispnéia com apresentação singular na tomografia computadorizada de tórax: síndrome de ativação macrofágica

Abstract

Macrophage activation syndrome is a rare and potentially life-threatening disease. It occurs due to immune dysregulation manifested as excessive macrophage proliferation, typically causing hepatosplenomegaly, pancytopenia and hepatic dysfunction. Here, we report an unusual case of macrophage activation syndrome presenting as dyspnea, as well as (reported here for the first time) high resolution computed tomography findings of an excavated nodule, diffuse ground glass opacities and consolidations (mimicking severe pneumonia or alveolar hemorrhage). The patient was successfully treated with human immunoglobulin. We recommend that macrophage activation syndrome be considered in the differential diagnosis of respiratory failure. Rapid diagnosis and treatment are essential to achieving favorable outcomes in patients with this syndrome.

Hemophagocytic Lymphohistiocytosis in Autopsied Adults: Clinical, Laboratory and Immunohistochemical Evaluation for CD68 and CD57. Case Report and Literature Review

“…23,30 It has already been described that the most relevant characteristic in HLH is a large number of macrophages phagocytizing hematopoietic cells in the bone marrow. 5,31 In the early stage of the disease, the bone marrow is generally hypercellular and contains few infiltrates of histiocytes. In the late phase, the bone marrow is hypocellular and contains several histiocytic infiltrates.…”

“…The primary HLH is a sporadic familial autosomal recessive disease characterized by mutations in the perforin (PRF1) gene, Munc-13-4 (UNC13D) gene, syntaxin 11 (STX11) gene, gene encoding syntaxin binding protein 2 (STXBP-2), SH2 domain-containing protein 1A (SH2D1A), and X-linked inhibitor of apoptosis protein (XIAP). [4][5][6][7] The secondary or reactive HLH is usually observed in adults and is associated with early stages of another disease such as infectious and malignant diseases, 8 chronic rheumatic diseases, 9 and juvenile dermatomyositis. 10 HLH is a hyper-inflammatory condition resulting from various disorders that affect macrophages, lymphocytes, and natural killer cells (NKC), 11 unleashing an excessive proliferation of those cells in response to the triggering agent.…”

“…10 HLH is a hyper-inflammatory condition resulting from various disorders that affect macrophages, lymphocytes, and natural killer cells (NKC), 11 unleashing an excessive proliferation of those cells in response to the triggering agent. 5,11,12 It is believed that there is an inappropriate control of cellular immune response and a decreased cytotoxic activity of NKC and CD8+ T cells. Consequently, antigen removal process is impaired, which stimulates the proliferation and activation of T cells.…”

“…[13][14][15] The microscopic evaluation shows that the proliferation of histiocytes usually occurs to all hematopoietic organs 12 with subsequent phagocytosis of erythrocytes by macrophages, forming hemosiderin in the bone marrow, spleen and lymph nodes. 5,15 The proliferation of T cells and macrophages in HLH causes sudden onset fever, hepatosplenomegaly, lymphadenopathy, pancytopenia, hepatic dysfunction, [16][17][18] fatigue, weight loss, multiple organ failure, and disseminated intravascular coagulation (DIC). 19 Several patients have a fulminant progression, with mortality rates ranging from 22% to 59%.…”

Secondary Hemophagocytic Lymphohistiocytosis Complicated by a Cavitary Lung Lesion in a Kidney Transplant Recipient

“…Our case was complicated by the pulmonary lesion different from the typical complications observed in HLH. There have been only singular case reports describing somewhat similar changes in lungs in the course of hyperinflammatory syndromes [ 10 , 11 ].…”