Session Information
Session Type: Abstract Submissions (ACR)
Background/Purpose :
Hemophagocytic Lymphohistiocytosis (HLH) is a severe and acute inflammatory syndrome usually fatal without treatment. HLH includes a spectrum of diseases produced by the proliferation and activation of T-cells and macrophages. The macrophage activation syndrome (MAS) is a variant of secondary HLH that occurs in autoimmune diseases.
Methods:
A retrospective search was performed by reviewing medical reports with HLH diagnosed between December 2008 and June 2013 at the Donostia University Hospital, Spain. Inclusion criteria were to meet the diagnostic criteria for HLH and had a bone marrow biopsy with hemophagocytic cells. The qualitative variables were: etiologies, triggers, treatments, causes of death and causes of admission in Intensive Care Unit (ICU); and the dichotomous were: sex, fever, organomegaly, mortality and ICU admission. The quantitative variables were: age, laboratory findings, diagnosis delay (days from the admission to the bone marrow biopsy), hospital and ICU stay. The quantitative variables were showed with median and interquartile range (IR).
Results:
Thirteen patients (7 men) with median age of 54 years (IR 34-61) were studied. Table 1 shows demographic characteristics, aetiologies, triggers, hospital stay, diagnosis delay, treatments and mortality of patients with diagnosis of secondary HLH. The diagnostic delay median was 14 days (IR 9-18) and the median hospital stay was 38 days (RI 30-61). Patient 9 continued at the end of this paper. Six patients were admitted to the I.C.U. (patients 3, 4, 6, 7, 8 and 9), the main cause of diagnosis admission was multi-organ dysfunction syndrome (MODS). The overall mortality rate was 30.1%, died 3 due MODS (patients 6, 7 and 8) and 1 due massive haemoptysis (patient 11). The only clinical criterion common to all patients was fever. Patient 12 was the only one without organomegaly. The diagnostic criteria and other laboratory data of each patient are given in table 2. All patients had at least one bone marrow biopsy with hemophagocytics cells; patient 3 also had hemophagocytics cells in the ascitic fluid.
Conclusion:
1) Hemophagocytics cells not only can be found in bone marrow, lymph nodes and spleen, but also in other pathological body fluids such as ascitic fluid, being a simpler method that could be performed before the biopsy or in doubt of it .
2) The mortality may be influenced by the aetiology and trigger of the HLH, the delay in diagnosis and the delay of immunosuppressive treatment. Patients with hematologic diseases had worse prognosis.
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Table 1: Demographics caracteristics, aetiologies, triggers, evolution, treatments and final resolution of patients with secondary HLH in at Donostia University Hospital. |
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|
Id
|
Aetiologies
|
Probable triggers
|
A
|
S
|
H.S.
|
D.D.
|
Treatments
|
Final
|
|
Rheumatogical Diseases.
|
1
|
Onset of S.L.E.
|
The disease itself.
|
57
|
M
|
38 days
|
3 days
|
1) Immunoglobulins. 2) Steroids. 3) Cyclosporine.
|
Cure
|
|
2
|
A.S.D.
|
Pneumococcus, CMV, Herpes simplex and Parvovirus B19.
|
30
|
F
|
10 days
|
7 days
|
1) Steroids. 2) Cyclosporine.
|
Cure
|
|
|
3
|
Onset of A.S.D.
|
The disease itself.
|
35
|
F
|
37 days
|
14 days
|
1) Steroids. 2) Cyclosporine. 3) Anakinra.
|
Cure
|
|
|
4
|
Onset of S.L.E.
|
The disease itself.
|
16
|
M
|
120 days
|
18 days
|
1) Steroids. 2) Immunoglobulins. 3) Cyclosporine. 4) Anakinra.
|
Cure
|
|
|
Hemato-oncological Diseases.
|
5
|
Onset of non-Hodgkin B and T cells lymphoma. |
The disease itself.
|
34
|
M
|
22 days
|
6 days
|
1) Campath-CHOP. 2) RTX-CHOP.
|
Cure
|
|
6
|
Acute myeloid leukemia.
|
HLA allogeneic bone marrow transplant. |
65
|
M
|
100 days
|
9 days
|
1) Steroids. 2) Cyclosporine.
|
Death
|
|
|
7
|
Onset of splenic large B-cell lymphoma. |
The disease itself.
|
77
|
F
|
61 days |
31 days
|
1) Steroids. 2) Immunoglobulins. 3) Cyclosporine. 4) Anakinra. 5) Tocilizumab.
|
Death
|
|
|
8
|
Onset of extranodal NK cell lymphoma. |
Epstein Barr virus.
|
55
|
M
|
38 days
|
27 days
|
1) Steroids 2) Immunoglobulins. 3) CHOP.
|
Death
|
|
|
9
|
Onset of splenic large B-cell lymphoma. |
The disease itself.
|
54
|
F
|
140 days
|
14 days
|
1) Steroids. 2) Ciclosporina. 3) Anakinra. 4) Tocilizumab. 5) R-CHOP. |
Still admitted |
|
|
Infectious Diseases.
|
10
|
Sepsis due to Campylobacter jejuni.
|
The disease itself.
|
61
|
M
|
31 days
|
18 days
|
1) Immunoglobulins. 2) Steroids
|
Cure
|
|
11
|
Stage C3 H.I.V.
|
Pneumocistis jiroveci or/and the disease itself. |
30
|
F
|
17 days
|
12 days
|
1) Steroids 2) Immunoglobulins.
|
Death
|
|
|
Oncologic Disease.
|
12
|
Grade IV glioblastoma multiforme
|
Chemotherapy with temozolomide.
|
78
|
M
|
30 days
|
15 days
|
1) Immunoglobulins. 2) Steroids.
|
Cure
|
|
Unknown disease.
|
13
|
Unknown disease |
Unknown trigger |
40
|
F
|
56 days
|
18 days
|
1) Steroids. 2) Immunoglobulins. 3) Cyclosporine
|
Cure |
|
Id: identification, A: Age in years, S: Sex, H.S: Hospital stay, D.D: Diagnosis delay, S.L.E: Systemic Lupus Erythematosus, A.S.D: Adult`s Still Disease, CMV: Cytomegalovirus, RTX: Rituximab, NK: Natural Killers, H.I.V: Human Immunodeficiency Virus, CHOP: cyclophosphamide, hydroxydaunorubicin, oncovin and prednisone. M: Male, F: Female.
|
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Table 2: Analytical characteristics of patients with secondary HLH at the Donostia University Hospital.
|
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ID
|
Hb
|
Plt
|
Leu
|
Neu
|
Tg
|
Fbr
|
Fer
|
GOT
|
GPT
|
LDH
|
TB
|
INR
|
PT
|
FA
|
GGT
|
|
Rheumatogical Diseases.
|
|||||||||||||||
|
1
|
8.9 |
117000 |
1200 |
580 |
417 |
NA |
15300 |
1080 |
608 |
824 |
0.5 |
1.1 |
5.6 |
275 |
922 |
|
2
|
9.8 |
8000 |
2340 |
1130 |
254 |
212 |
2389 |
395 |
566 |
959 |
1.1 |
NA |
NA |
341 |
243 |
|
3
|
7.3 |
5600 |
3340 |
1006 |
604 |
103 |
175266 |
1318 |
596 |
7748 |
12.7 |
1.6 |
4.4 |
431 |
584 |
|
4
|
6.1 |
72000 |
1660 |
650 |
412 |
77 |
15359 |
512 |
199 |
993 |
5.2 |
2 |
4 |
297 |
681 |
|
Hemato-oncological Diseases. |
|||||||||||||||
|
5
|
7.4 |
22000 |
820 |
360 |
382 |
NA |
NA |
86 |
104 |
259 |
0.8 |
1.3 |
4.3 |
231 |
114 |
|
6
|
5.9 |
2000 |
0 |
0 |
340 |
NA |
16796 |
277 |
385 |
352 |
11.5 |
1.4 |
3.9 |
262 |
1132 |
|
7
|
7 |
16000 |
50 |
0 |
227 |
287 |
2868 |
175 |
270 |
1120 |
2.5 |
4.7 |
4 |
142 |
266 |
|
8
|
7.4 |
17000 |
1300 |
2000 |
427 |
103 |
53695 |
199 |
108 |
1145 |
3.4 |
1.05 |
4.7 |
435 |
1162 |
|
9
|
6.6 |
4000 |
10 |
0 |
1134 |
670 |
16705 |
419 |
147 |
2490 |
12.4 |
1.57 |
4.3 |
811 |
1062 |
|
Infectious Diseases. |
|||||||||||||||
|
10
|
7.8 |
9000 |
1010 |
0 |
471 |
214 |
10938 |
134 |
234 |
441 |
1.7 |
1.1 |
5.6 |
73 |
96 |
|
11
|
7.3 |
10000 |
3090 |
2320 |
274 |
NA |
9755 |
23 |
32 |
752 |
1.6 |
1.4 |
5.4 |
172 |
227 |
|
Oncologic Disease. |
|||||||||||||||
|
12
|
8.6 |
5000 |
480 |
230 |
149 |
NA |
NA |
54 |
88 |
426 |
3.7 |
1.9 |
3.1 |
68 |
173 |
|
Unknown Disease |
|||||||||||||||
|
13
|
7.5 |
15000 |
5790 |
3030 |
561 |
513 |
2422 |
50 |
46 |
564 |
1.1 |
1.48 |
4.6 |
407 |
491 |
|
Median
|
7.4 |
10000 |
1200 |
580 |
412 |
212 |
15300 |
199 |
199 |
824 |
2.5 |
1.46 |
4.35 |
275 |
491 |
|
P25
|
7 |
5600 |
480 |
0 |
274 |
103 |
6311.5 |
86 |
104 |
441 |
1.1 |
1.26 |
4 |
172 |
227 |
|
P75
|
7.8 |
17000 |
2340 |
1130 |
471 |
287 |
16750.5 |
419 |
385 |
1120 |
5.2 |
1.64 |
4.88 |
407 |
922 |
|
Hb: hemoglobin (mg/dl). Plt: platelets (ul). Neu: neutrophils (/ul). Tg: triglycerides (mg/dl). Fbr: fibrinogen (mg/dl). Fer: Ferritin (ng/ml). GOT Glutamic oxalacetic transaminase (mg/dl). GPT: Glutamic-pyruvic transaminase (mg/dl) LDH: Lactate dehydrogenase (IU/l). TB: Total bilirubin (mg/dl). INR: International normalized ratio TP: Total proteins (g/dl). ALP: Alkaline phosphatase (mg/dl). GGT: Gamma glutyl transferase (IU/L). NA: No test performed. |
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Disclosure:
C. A. Egües Dubuc,
None;
V. Aldasoro Cáceres,
None;
M. Uriarte Ecenarro,
None;
C. Meneses Villalba,
None;
N. Errazquin Aguirre,
None;
I. Hernando Rubio,
None;
O. Maiz Alonso,
None;
J. Cancio Fanlo,
None;
E. Uriarte Isacelaya,
None;
J. Belzunegui Otano,
None.
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