A Very Rare Case of Secondary Hemophagocytic Lymphohistiocytosis in an AIDS Patient With Refractory Septic Shock and Overwhelming Disseminated Histoplasmosis

CHEST Journal ◽  
2013 ◽  
Vol 144 (4) ◽  
pp. 287A
Author(s):  
Bassam Yaghmour ◽  
Ivan Romero-Legro ◽  
Paul Bryant ◽  
Luis Murillo ◽  
Amado Freire
Keyword(s):  
Septic Shock ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Rare Case ◽  
Disseminated Histoplasmosis ◽  
Refractory Septic Shock ◽  
Secondary Hemophagocytic Lymphohistiocytosis

scholarly journals Hemophagocytic syndrome masquerading as septic shock: An approach to such dilemma

2017 ◽  
Vol 5 ◽  
pp. 2050313X1774630 ◽  
Author(s):  
Zakaria Hindi ◽  
Abdallah A Khaled ◽  
Ashraf Abushahin
Keyword(s):  
Septic Shock ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Hemophagocytic Syndrome ◽  
Specific Treatment ◽  
Rare Condition ◽  
Unknown Origin ◽  
Chronic Obstructive ◽  
Obstructive Pulmonary Disease ◽  
Focus Of Infection ◽  
Refractory Septic Shock

Introduction: Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis is a rare condition characterized by excessive inflammation that is thought to be caused by the absence of normal downregulation of activated macrophages and lymphocytes. The treatment of hemophagocytic lymphohistiocytosis can depend on whether it is primary or secondary. In secondary hemophagocytic lymphohistiocytosis, the treatment can be directed according to the cause. In general, protocol HLH-94 (which consists of dexamethasone and etoposide in induction and maintenance) has been widely used as it has good outcomes. Hemophagocytic lymphohistiocytosis and septic shock largely overlap which can lead to refractory septic shock and death if not treated. Unfortunately, there is no clear approach for such dilemma. Thereby, we would like to present our case as it has a valuable approach to hemophagocytic lymphohistiocytosis in the setting of sepsis. Case description: A 60-year-old female, with history of hypertension, came with fever, productive cough, and dyspnea; she was admitted for acute exacerbation of chronic obstructive pulmonary disease and was transferred to intensive care unit for septic shock. The patient progressed to refractory septic shock with no focus of infection. After further investigations, detailed history raised the suspicion of hemophagocytic lymphohistiocytosis; a bone marrow biopsy was collected and confirmed the diagnosis. The patient was on methylprednisolone while waiting for other investigation results and improved markedly. After ruling out secondary causes of hemophagocytic lymphohistiocytosis, she was switched to protocol-94 and continued to improve. Conclusion: It should be emphasized that septic shock, with or without focus of infection, overlaps with hemophagocytic lymphohistiocytosis and can consequently lead to refractory septic shock and death. Thus, our aim of this case is to encourage further investigations, specifically for hemophagocytic lymphohistiocytosis in the setting of septic shock of unknown origin, to decrease mortality rate. More importantly, early initiation of immunosuppression therapy may be a crucial step before switching to hemophagocytic lymphohistiocytosis–specific treatment.

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