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