Autopsy findings in 27 children with haemophagocytic lymphohistiocytosis

Abstract

The diagnosis of HLH must be based on clinical, histological and additional laboratory findings. A negative bone marrow examination is common. Previous treatment with steroids and/or cytostatic drugs may attenuate or even eliminate the typical histological findings. Liver findings similar to those in chronic persistent hepatitis are common.

Unexpected Thrombocytopenia and Anemia in Cynomolgus Monkeys Induced by a Therapeutic Human Monoclonal Antibody

“…Histologically, lymphocytes and mature macrophages exhibiting hemophagocytosis accumulate in numerous organs including spleen, liver, lymph nodes, bone marrow, and central nervous system (Ost, Nilsson-Ardnor, and Henter 1998). The clinical (Henter, Carlson, et al 1991) and hypofibrinogenemia results from fibrinolysis due to the release of plasminogen activator.…”

Macrophage activation syndrome and other systemic inflammatory conditions after BMT

“…4 A bone marrow examination is not required to establish the diagnosis, and autopsy findings of marrow hemophagocytosis are actually present in a minority of children at the time of death. 11 Classically, FHL manifests in the first few months of life, although rare cases have been reported with onset in adult years. 12 Three new diagnostic criteria have been added by the Histiocyte Society: (1) low or absent natural killer (NK) cell activity, (2) hyperferritinemia (ferritin X500 mg/l and (3) high plasma CD25 (IL-2 receptor) X2400 U/ml.…”

Haemophagocytic lymphohistiocytosis in Adult- A Case Report and Literature Review

“…53,4,54 Autopsy evaluation study of the liver has shown chronic persistent hepatitis with periportal lymphocytic infiltration in 22 of 27 patients with HLH. 56 Our reported case had transaminitis. Hypertriglyceridemia, hypofibrinogenemia, elevated serum lactate dehydrogenase, hyponatremia are frequently seen in HLH.…”

“…As a result, if the bone marrow is not conclusive, material should be obtained from other organs e.g. ; liver, spleen, and lymph nodes 60,56 and occasionally the central nervous system , 61, 62 skin 63 and serial aspirates over time may also be helpful . 58,59 In our case, the bone marrow aspiration was performed and it revealed haemophagocytosis.…”

“…Haemophagocytosis may be present in the liver and infiltration of the hepatic portal tracts with lymphocytes is also common. 60,56 Although haemophagocytosis in bone marrow is associated with HLH, the morphologic phenomenon may also be induced by more common events, including blood transfusions, infection, autoimmune disease, and other forms of bone marrow failure or causes of red blood cell destruction . [64][65][66] Despite the nomenclature of HLH, diagnosis should never be made or excluded solely on the presence or absence of haemophagocytosis.…”