Prominent dyserythropoiesis in four cases of haemophagocytic lymphohistiocytosis.
Haemophagocytic lymphohistiocytosis (HLH) is a disease characterised by peripheral blood pancytopenia secondary to haemophagocytosis of formed blood cells by activated histiocytes. The demonstration of haemophagocytosis may be difficult and the diagnosis may require repeated tissue sampling (including bone marrow, cerebrospinal fluid, lymph nodes, spleen, and liver) and the demonstration of associated clinical or laboratory features. This report describes pronounced dyserythropoiesis in the bone marrow aspirates in four patients with HLH, including familial and secondary cases. In three patients, bone marrow haemophagocytosis was inconspicuous or absent, and the prominent dyserythropoiesis may have suggested an alternative diagnosis. The dyserythropoiesis observed should be added to the constellation of clinical and laboratory features associated with HLH.
Macheta
M
M
Department of Paediatric Haematology, Royal Manchester Children's Hospital, Hospital Road, Pendlebury, Manchester M27 4HA, UK.
Will
A M
AM
Houghton
J B
JB
Wynn
R F
RF
D000328:Adult; D001853:Bone Marrow / Q000473:pathology*; D016026:Bone Marrow Transplantation; D003131:Combined Modality Therapy; D003907:Dexamethasone / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D004920:Erythropoiesis; D005047:Etoposide / Q000627:therapeutic use; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D015616:Histiocytosis, Non-Langerhans-Cell / Q000188:drug therapy / Q000473:pathology* / Q000628:therapy; D006801:Humans; D007223:Infant; D008297:Male; D019384:Nucleic Acid Synthesis Inhibitors / Q000627:therapeutic use
D005938:Glucocorticoids; D019384:Nucleic Acid Synthesis Inhibitors; D005047:Etoposide; D003907:Dexamethasone