open access
Clinicomorphological spectrum of hemophagocytic syndrome in a tertiary care hospital


- PSG Institute of Medical Sciences & Research, Coimbatore, India
open access
Abstract
Introduction: The HLH-2004 trial established the diagnostic criteria for hemophagocytic lymphohistiocytosis (HLH), a severe hyperinflammatory condition. It typically develops due to inappropriate macrophage activation. Our objective was to assess the spectrum of hemophagocytic syndrome presentations by identifying hemophagocytic activity in the bone marrow, and to unravel the etiopathogenesis of this condition. Material and methods: A retrospective study was carried out in the Department of Pathology in a tertiary care hospital reporting the clinical and laboratory findings of patients who had been previously diagnozed with hemophagocytosis in the bone marrow. The parameters in the diagnostic criteria of HLH of the same patients were documented and analyzed. Results: The characteristics of the 32 patients who presented with hemophagocytosis in the bone marrow were documented. Persistent fever was the most frequent presentation. Mild to moderate anemia (69%), severe leucopenia (59%), and mild to moderate thrombocytopenia (63%) were other frequent findings. The incidence of primary HLH was found to be only 3%; 87% had hyperferritinemia, 78% had bicytopenia, 59% had hypertriglyceridemia, and 53% had splenomegaly. Infections followed by malignancies were shown to be the most frequent cause of secondary HLH, while the prognosis for malignancy-associated HLH appeared to be poor. Conclusions: Based on the findings of this study, conclusions about the clinical symptoms and etiologies of HLH may be drawn, which will assist in early identification. Hence, all subjects with a clinical suspicion of HLH should be thoroughly investigated for a possible etiology.
Abstract
Introduction: The HLH-2004 trial established the diagnostic criteria for hemophagocytic lymphohistiocytosis (HLH), a severe hyperinflammatory condition. It typically develops due to inappropriate macrophage activation. Our objective was to assess the spectrum of hemophagocytic syndrome presentations by identifying hemophagocytic activity in the bone marrow, and to unravel the etiopathogenesis of this condition. Material and methods: A retrospective study was carried out in the Department of Pathology in a tertiary care hospital reporting the clinical and laboratory findings of patients who had been previously diagnozed with hemophagocytosis in the bone marrow. The parameters in the diagnostic criteria of HLH of the same patients were documented and analyzed. Results: The characteristics of the 32 patients who presented with hemophagocytosis in the bone marrow were documented. Persistent fever was the most frequent presentation. Mild to moderate anemia (69%), severe leucopenia (59%), and mild to moderate thrombocytopenia (63%) were other frequent findings. The incidence of primary HLH was found to be only 3%; 87% had hyperferritinemia, 78% had bicytopenia, 59% had hypertriglyceridemia, and 53% had splenomegaly. Infections followed by malignancies were shown to be the most frequent cause of secondary HLH, while the prognosis for malignancy-associated HLH appeared to be poor. Conclusions: Based on the findings of this study, conclusions about the clinical symptoms and etiologies of HLH may be drawn, which will assist in early identification. Hence, all subjects with a clinical suspicion of HLH should be thoroughly investigated for a possible etiology.
Keywords
bone marrow, hemophagocytic lymphohistiocytosis (HLH), hyperferritinemia, primary HLH, secondary HLH




Title
Clinicomorphological spectrum of hemophagocytic syndrome in a tertiary care hospital
Journal
Issue
Article type
Original research article
Pages
36-42
Published online
2023-02-02
Page views
430
Article views/downloads
44
DOI
10.5603/AHP.a2023.0005
Bibliographic record
Acta Haematol Pol 2023;54(1):36-42.
Keywords
bone marrow
hemophagocytic lymphohistiocytosis (HLH)
hyperferritinemia
primary HLH
secondary HLH
Authors
Abinaya Sundari
Prasanna N Kumar


- Somasundaram V, Srinivasagowda S, Balraam V, et al. Hemophagocytosis in bone marrow aspirates: an indication of hidden pathologies. Med J DY Patil Vidyapeeth. 2019; 12(1): 22–27.
- Chandra H, Chandra S, Kaushik Rm, et al. Hemophagocytosis on bone marrow aspirate cytology: single center experience in north himalayan region of India. Ann Med Health Sci Res. 2014; 4(5): 692–696.
- Bami S, Vagrecha A, Soberman D, et al. The use of anakinra in the treatment of secondary hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2020; 67(11): e28581.
- Campo M, Berliner N. Hemophagocytic lymphohistiocytosis in adults. Hematol Oncol Clin North Am. 2015; 29(5): 915–925.
- Shah AR, Muzzafar T, Assi R, et al. Hemophagocytic lymphohistiocytosis in adults: an under recognized entity. BBA Clin. 2017; 7: 36–40.
- Gars E, Purington N, Scott G, et al. Bone marrow histomorphological criteria can accurately diagnose hemophagocytic lymphohistiocytosis. Haematologica. 2018; 103(10): 1635–1641.
- Lehmberg K, Nichols KE, Henter JI, et al. Study Group on Hemophagocytic Lymphohistiocytosis Subtypes of the Histiocyte Society. Consensus recommendations for the diagnosis and management of hemophagocytic lymphohistiocytosis associated with malignancies. Haematologica. 2015; 100(8): 997–1004.
- Grzybowski B, Vishwanath VA. Hemophagocytic lymphohistiocytosis: a diagnostic conundrum. J Pediatr Neurosci. 2017; 12(1): 55–60.
- Hayden A, Park S, Giustini D, et al. Hemophagocytic syndromes (HPSs) including hemophagocytic lymphohistiocytosis (HLH) in adults: A systematic scoping review. Blood Rev. 2016; 30(6): 411–420.
- Otrock ZK, Eby CS. Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis. Am J Hematol. 2015; 90(3): 220–224.
- Kim YuRi, Kim DY. Current status of the diagnosis and treatment of hemophagocytic lymphohistiocytosis in adults. Blood Res. 2021; 56(S1): S17–S25.
- Iqbal W, Alsalloom AA, Shehzad K, et al. Hemophagocytic histiocytosis: a clinicopathological correlation. Int J Health Sci (Qassim). 2017; 11(1): 1–7.
- George MR. Hemophagocytic lymphohistiocytosis: review of etiologies and management. J Blood Med. 2014; 5: 69–86.
- Fardet L, Galicier L, Lambotte O, et al. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol. 2014; 66(9): 2613–2620.
- Verma N, Chakraverty J, Baweja P, et al. Extremely high ferritinemia associated with haemophagocytic lympho histiocytosis (HLH). Indian J Clin Biochem. 2017; 32(1): 117–120.
- Ho C, Yao X, Tian L, et al. Marrow assessment for hemophagocytic lymphohistiocytosis demonstrates poor correlation with disease probability. Am J Clin Pathol. 2014; 141(1): 62–71.
- Zhang L, Zhou J, Sokol L. Hereditary and acquired hemophagocytic lymphohistiocytosis. Cancer Control. 2014; 21(4): 301–312.
- Non LR, Patel R, Esmaeeli A, et al. Typhoid fever complicated by hemophagocytic lymphohistiocytosis and rhabdomyolysis. Am J Trop Med Hyg. 2015; 93(5): 1068–1069.
- Hust MA, Blechacz BRA, Bonilla DL, et al. Adult cancer-related hemophagocytic lymphohistiocytosis — a challenging diagnosis: a case report. J Med Case Rep. 2017; 11(1): 172.
- Sundari AA. Clinicopathological spectrum of haemophagocytic syndrome. Masters thesis. PSG Institute of Medical Sciences and Research, Coimbatore 2018. http://repository-tnmgrmu.ac.in/9451/1/200301218abinaya_sundari.pdf (September 8, 2018).