Surviving acquired severe nonmalignant hemophagocytic syndrome

Background: Hemophagocytic syndrome (HPCS) is not rare in Thailand. At present the outcome in severe cases is often unsatisfactory because of a delay in diagnosis and improper management. From 1999 to 2011, the authors have gained experience in the management of severe acquired nonmalignant HPCS in Thai adult patients. From 1999 to 2003, three out of 10 patients died. During 2005-2011, seven patients with severe HPCS survived. This improvement stimulated the authors to analyze clinical manifestations, etiology, and management of these patients.

Objective: To find the keys to successful outcomes in the management of acquired severe nonmalignant HPCS in seven survivors during 2005-2011. To alert physicians that acquired severe nonmalignant HPCS may be cured.

Materials and Methods: The records of ten patients with HPCS were analyzed. Between 1999 and 2003 three patients died. Between 2005 and 2011 seven patients survived.

Results: The three patients during 1991-2003 may well have died because of delayed diagnosis and improper management. One case of HPCS was diagnosed after death. In the other two cases etiologies were not readily identified and dealt with. In the surviving group, treatment was started as soon as HPCS was suspected or diagnosed. This consisted of removal of any known cause and aggressive management of associated conditions and complications.

Conclusion: Adults who acquire severe nonmalignant HPCS can be treated successfully. The key roles for this success are early diagnosis and appropriate management. The best supportive care and close observation by the team of hematologists and physicians also determines patient survival.