Acquired hemophagocytic lymphohistiocytosis associated with multiple myeloma.
Acquired or secondary hemophagocytic lymphohistiocytosis (sHLH) is a life-threatening hyperinflammation syndrome caused mostly by various infectious agents, autoimmune disorders or malignancy. So far, only anecdotal cases of sHLH associated with multiple myeloma have been published. We provide a review of all these reports and include a previously not published case of myeloma-associated sHLH in a 59-year-old male with complex partial epilepsy. Due to aggressive course of sHLH, increased awareness is indicated in all patients with malignancies which develop unremitting fever, cytopenia and splenomegaly. Early diagnosis and immediate introduction of adequate therapy is crucial for the outcome of HLH.
Machaczka
Maciej
M
Hematology Center Karolinska, M54, Karolinska University Hospital Huddinge, Stockholm, Sweden. maciej.machaczka@ki.se
Vaktnäs
Johan
J
Klimkowska
Monika
M
Nahi
Hareth
H
Hägglund
Hans
H
D000927:Anticonvulsants / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D004827:Epilepsy / Q000150:complications / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications* / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D009101:Multiple Myeloma / Q000150:complications* / Q000628:therapy; D011241:Prednisone / Q000627:therapeutic use; D033581:Stem Cell Transplantation
D000927:Anticonvulsants; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011241:Prednisone