Hemophagocytic lymphohistiocytosis complicated by multiorgan failure

Abstract

Rationale:We present a case of hemophagocytic lymphohistiocytosis (HLH) with severe pulmonary complication and acute respiratory distress syndrome (ARDS) hospitalized in our intensive care unit (ICU) in 2014; distinctive trait of this case has been the challenging diagnosis, with a bone marrow biopsy always negative, the severe pulmonary complication with ARDS and severe pulmonary hypertension, and the ferritin temporal kinetics that precisely followed the clinical course of disease.Patient concerns:A 32-year-old woman from the Philippines first diagnosed with upper airway infection, was subsequently hospitalized in infectious disease department and treated for community acquired pneumonia.Diagnoses:After clinical picture worsened with a profound respiratory insufficiency, the patient was intubated and transferred to our ICU. During this hospitalization, the clinical picture of fever, cutaneous rashes, lymphadenitis, hepatitis, leukopenia, anemia, hyperferritinemia, hypertriglyceridemia, high level of auto-antibodies, and low NK activity suggested an hemophagocytic lymphohistiocytosis syndrome, even if bone marrow biopsy was negative for hemophagocytosis.Interventions:Immunosuppressive therapy with dexamethasone and etoposide was started, and the patient was discharged from ICU 4 months after admission.Lessons:HLH is a rare disorder of the mononuclear phagocytic system, characterized by systemic proliferation of non- neoplastic histiocytes. The diagnosis is often challenging and not all of the diagnostic criteria may be present at the same time; this case shows how complex the diagnosis could be, how hematic ferritin levels could help in following the course of the disease, and the possibility of severe pulmonary complication either due to the disease itself and to possible sovra infections.

Management of Hemophagocytic Lympho-Histiocytosis in Critically Ill Patients

“…Similarly, HS should be considered as a possible diagnosis for patients admitted to the ICU with septic shock 11 and preexisting fever and asthenia for several weeks. [12][13][14] Asthenia is usually associated. 9 Nonregenerative anemia is the most frequent hematological sign (90%-100%).…”

“…This remains the most difficult part of the diagnosis. 8,13,14 When there is a clinical and biological suspicion of HS, clinicians should perform bone marrow aspiration. Hemophagocytic figures are very frequent ( Figure 2) but not constant.…”

“…Without treatment, HS can lead to organ failures requiring ICU admission. 5,8,14 Only few studies described in detail the organ failures in patients admitted to ICU with suspicion of HS. One retrospective study described 75 patients who fulfilled HS criteria and were admitted to the ICU.…”

Development of a dendrimer PAMAM‑based gold biochip for rapid and sensitive detection of endogenous IFN‑γ and anti‑IFN‑γ IgG in patients with hemophagocytic lymphohistiocytosis

“…Autoantibodies, antigens and inflammatory mediators can trigger inflammation (29,30). Although circulating autoantibodies have been detected in patients with HlH (29,31), to the best of our knowledge, there have been no reports of endogenous anti-iFn-γ igG production in the context of HlH pathogenesis. in preclinical trials, neutralizing Figure 6.…”

Marchiafava-Bignami disease with haemophagocytic lymphohistiocytosis as a postoperative complication of cardiac surgery

“…Cerebrospinal fluid analysis on POD 10 showed the following: cell count <1/mm 3 ; protein, 38 mg/dL; glucose, 93 mg/dL (CSF-blood glucose ratio, 0.49); myelin basic protein (MBP), 4120 pg/mL (reference values: <102 pg/mL); and negative findings for bacterium and malignancy. Although, intermittent or not simultaneous, continued fever over 40°C, clinical history of bicytopenia, significantly elevated AST, ALT and LDH, low natural killer cell activity, hyperferritinoaemia (3006.6 ng/mL) (reference values: 21.8–274.6 ng/mL), hypofibrinogenaemia and even ARDS potentially suggested haemophagocytic lymphohistiocytosis (HLH) 5. We also performed a bone mallow aspiration on POD 65, although it was after steroid therapy.…”