8/21/2023 0 Comments Hlh up to date![]() Symptoms of hemophagocytic lymphohistiocytosis (including fever, malaise, myalgia, etc.Hemophagocytosis is not a specific finding and can be seen in up to 60% of patients with sepsis ( Clin Case Rep 2018 6:2466).Renal disease (malignant hypertension, glomerulonephritis, pyelonephritis).Trauma to lymph drainage sites (i.e., following breast biopsy).Enlarged lymph nodes (especially of the axilla) displaying prominent erythrophagocytosis may not be indicative of systemic HLH but rather can be observed in the setting of.Later stages demonstrate lymphoid depletion with massive sinusoidal infiltration by benign appearing histiocytes and hemophagocytosis.Early disease shows an intense immunoblastic response.Lymph node involvement in systemic HLH features.Splenic white pulp can be atrophic with extensive infiltration of histiocytes exhibiting hemophagocytosis.Liver sinusoids can demonstrate histiocytes with hemophagocytosis.Hemophagocytic cells are best visualized on bone marrow aspirate smear.Hemophagocytosis (histiocyte / macrophage ingestion of erythrocytes, leukocytes, platelets or their precursor cells), including erythrophagocytosis (ingestion of erythrocytes).In adults, hemophagocytic lymphohistiocytosis should prompt an investigation for underlying malignancy.In children, a genetic defect characteristic of FHLH should be ruled out.Pregnancy / HELLP syndrome (rare) ( Clin Case Rep 2018 6:2466). ![]() Malignancy (most often NK / T cell neoplasms, such as panniculitis-like T cell lymphoma, nasal type extranodal NK / T cell lymphoma and cutaneous gamma / delta T cell lymphoma). ![]()
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