Efficient management of secondary haemophagocytic lymphohistiocytosis with intravenous steroids and γ-immunoglobulin infusions

doi:10.12998/wjcc.v7.i21.3394

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World Journal of Clinical Cases

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Baishideng Publishing Group Inc, 7041 Koll Center Parkway, Suite 160, Pleasanton, CA 94566, USA

Retrospective Study

World J Clin Cases. Nov 6, 2019; 7(21): 3394-3406
Published online Nov 6, 2019. doi: 10.12998/wjcc.v7.i21.3394

Table 1 Characteristics of the 80 adult patients with secondary haemophagocytic lymphohistiocytosis

Characteristic	n (%)
Clinical symptoms
Fever	75 (94)
Respiratory symptoms	24 (30)
Skin rash	20 (25)
Gastrointestinal symptoms	8 (10)
CNS symptoms¹	7 (9)
Physical findings
Splenomegaly	47 (59)
Hepatomegaly	47 (59)
Lymphadenopathy	37 (46)
Laboratory findings
Elevated ferritin (≥ 500 ng/mL)	76 (95)
(≥ 10000 ng/mL)	20 (25)
Elevated inflammatory markers²	69 (86)
Complete blood count
Anaemia	58 (72)
Thrombocytopenia	57 (71)
Leucopoenia/neutropenia	38 (47)/9 (11)
Pancytopenia	24 (30)
Elevated LFTs	70 (87)
Elevated LDH	61 (76)
High triglycerides	55 (70)
Low fibrinogen levels	18 (22)
DIC	9 (11)

¹Disturbance of consciousness, headache/dizziness.

²C-reactive protein and erythrocyte sedimentation rate. CNS: Central nervous system; LFTs: Liver function tests; LDH: Lactate dehydrogenase; DIC: Disseminated intravascular coagulation.

Table 2 Underlying disease in the 80 patients with secondary haemophagocytic lymphohistiocytosis

Disease	n
Infection
Parasite
Leishmania spp. alone	15
Leishmania spp. plus Coxiella Burnetti	1
Bacteria
Coxiella Burnetti alone	3
Staphylococcus aureus	2
Acinetobacter baumannii plus Achromobacter xylosoxidans	1
Acinetobacter baumannii plus Aspergillus spp.	1
Achromobacter xylosoxidans alone	1
E. Coli	2
Pseudomonas aeruginosa	1
Rickettsia spp.	1
Brucella spp.	1
Virus
EBV	7
Influenza (A/H3N2)	3
HSV	1
CMV¹	1
Measles	1
Fungi
Aspergillus spp.	1
Candida spp.	1
Mycobacteria
Mycobacterium Fortuitum	1
Unidentified pathogen	12
Neoplasia
Non-Hodgkin lymphoma	10
Non-Hodgkin lymphoma accompanied by Leismaniasis or Coxiella Burnetti	2
Solid tumour	1
Autoimmune disease
Adult Still’s disease	5
Psoriatic arthritis	1
Other
Toxic epidermal necrolysis	1
Wilson disease	1
Unknown	2

¹The patient was also positive for human immunodeficiency virus. EBV: Epstein-Barr virus; HSV: Herpes simplex virus; CMV: Cytomegalovirus.

Citation: Georgiadou S, Gatselis NK, Stefos A, Zachou K, Makaritsis K, Rigopoulou EI, Dalekos GN. Efficient management of secondary haemophagocytic lymphohistiocytosis with intravenous steroids and γ-immunoglobulin infusions. World J Clin Cases 2019; 7(21): 3394-3406
URL: https://www.wjgnet.com/2307-8960/full/v7/i21/3394.htm
DOI: https://dx.doi.org/10.12998/wjcc.v7.i21.3394