Acute Monoblastic Leukemia (AML-M5) with Concomitant Hemophagocytic Lymphohistiocytosis: Presenting as Pancytopenia

Main Article Content

Sonal .
Deepti Gupta
Nupur Trivedi
Shefali Agarwal
Archana Agarwal
Anjali Tewari
Priyanka Verma


Hemophagocytic Lymphohistiocytosis (HLH) is an aggressive hyperinflammatory condition characterized by prolonged fever, cytopenias, hepatosplenomegaly and hemophagocytosis with a high rate of mortality. Its diagnosis is a challenge for pathologists as well as clinicians due to variable overlap of symptoms with other severe diseases. AML-associated hemophagocytic lymphohistiocytosis is a rare event, reported only in limited members.

We, hereby report a case of 14 year old male child who came to our hematology department with a history of prolonged fever, pancytopenia and 40 transfusions in last one year. Peripheral blood counts showed 15% blastoid atypical cells. Bone marrow revealed acute leukaemia with evidence of haemophagocytosis. A diagnosis of AML-M5 with HLH was made after flow cytometry and completion of clinico-biochemical criteria for HLH.

Patients with HLH in acute leukaemia may have low blast counts on peripjeral blood leading to much delay in the diagnosis. This case report provides helpful clinical experience related to diagnosis of this rare entity.

Hemophagocytic Lymphohistocytosis (HLH), hyperinflammatory condition, AML-associated, secondary HLH, cytopenias.

Article Details

How to Cite
., S., Gupta, D., Trivedi, N., Agarwal, S., Agarwal, A., Tewari, A., & Verma, P. (2020). Acute Monoblastic Leukemia (AML-M5) with Concomitant Hemophagocytic Lymphohistiocytosis: Presenting as Pancytopenia. Asian Hematology Research Journal, 2(4), 1-5. Retrieved from
Case Study


Janka G, Henter JI, Imashuku S. Clinical aspects and therapy of hemophagocytic lymphohistiocytosis. Cambridge UK: Cambridge University Press; 2005.

Janka G, Imashuku S, Elinder G, Schneider M, Henter JI. Infection-and malignancy-associated hemophagocytic syndromes: Secondary hemophagocytic lymphohistiocytosis. Hematology/oncology clinics of North America. 1998;12(2):435-44.

Akyay A, Celkan T, Iskender G, Tukun A, Ogan C, Olcay L. Two acute myeloblastic leukemia cases concomitant with hemophagocytic lymphohistiocytosis and review of the literature. Annals of Clinical & Laboratory Science. 2013;43(1):85-90.

Janka GE. Hemophagocytic syndromes. Blood reviews. 2007;21(5):245-53.

Szyper-Kravitz M. The hemophagocytic syndrome / macrophage activation syndrome: A final common pathway of a cytokine storm. The Israel Medical Association Journal. 2009;11(10):633.

Komp DM, McNamara J, Buckley P. Elevated soluble interleukin-2 receptor in childhood hemophagocytic histiocytic syndromes. Blood. 1989;73(8):2128- 32.

Ramos-Casals M, Brito-Zeron P, Lopez-Guillermo A, Khamashta MA, Bosch X. Adult haemophagocytic syndrome. The Lancet. 2014;383(9927):1503-16.

Ishii E, Ohga S, Imashuku S, Yasukawa M, Tsuda H, Miura I, Yamamoto K, Horiuchi H, Takada K, Ohshima K, Nakamura S. Nationwide survey of hemophagocytic lymphohistiocytosis in Japan. International journal of hematology. 2007;86(1):58-65.

Majluf-Cruz A, Sosa-Camas R, Perez-Ramirez O, Rosas-Cabral A, Vargas-Vorackova F, Labardini-Mendez J. Hemophagocytic syndrome associated with hematological neoplasms. Leukemia research. 1998;22(10):893-8.

O'Brien MM, Lee‐Kim Y, George TI, McClain KL, Twist CJ, Jeng M. Precursor B‐cell acute lymphoblastic leukemia presenting with hemophagocytic lymphohistiocytosis. Pediatric blood & cancer. 2008;50(2):381-3.

Meki A, O'Connor D, Roberts C, Murray J. Hemophagocytic lymphohistiocytosis in chronic lymphocytic leukemia. Journal of Clinical Oncology. 2011;29(24):e685-7.

Jordan MB, Allen CE, Weitzman S, Filipovich AH, McClain KL. How I treat hemophagocytic lymphohistiocytosis. Blood. 2011;118(15):4041-52.