Macrophage Activation Led Acute Heart Failure Managed Successfully with Immunosuppression

Figures & data

Table 1 Literature Review of Prior Cases with HLH Systolic Heart Failure

Case	Age	Association	Takotsubo Features	Treatment	Complete Recovery of Cardiac Function on Echo
Zhou JY, et al^Citation14	45-year-old female	Lamotrigine	Yes	Etoposide, dexamethasone	Yes
Otillio JK, et al^Citation15	6-year-old female	Infection	Yes	Etoposide, cyclosporine, and dexamethasone	Yes
Takeoka Y, et al^Citation16	73-year-old (A) and 56- year-old female (B)	EBV (A), T cell lymphoma (B)	Yes (A and B)	Etoposide, cyclosporine, and dexamethasone (A); carboplatin, cytarabine, dexamethasone (B)	Yes (A and B)
Prakash PB, et al^Citation3	1-year old female	Infection	No	(anakinra), methylprednisolone and tacrolimus	Yes
Abdin A^Citation17	59-year-old male	Unclear etiology	Yes	High-dose steroids	Yes
Ullah W, et al^Citation18	32-year-old male	Infection (HIV)	Yes	Dexamethasone, etoposide	Yes
Chizinga M, et al^Citation9	32-year-old male	Systemic juvenile idiopathic arthritis (SJIA)	No (right sided heart failure)	Dexamethasone, Anakinra.	Not reported, patient clinically improved

Table 2 Laboratory, Bone Marrow and ECHO Findings of Case 1 and 2

	Case 1	Case 2
Laboratory
Hb (11.6–15)	9.3 g/dL	7.9 g/dL
WCC (3.4–9.6) x10^9	2.4 /L	1.1 /L
Platelet (157–371)	76 /L	33 /L
Triglyceride	556 mg/dL	970 mg/dL
Ferritin (11–307)	731 mcg/L	5446 mcg/L
LDH (122–222)	261 U/L	534 U/L
IL 2 receptor (CD25) (≤1033)	—–	1599 pg/mL
CRP (<8.0)	92.4 mg/L	—–
IL-6 (<1.8)	8.6 pg/mL	10.2 pg/mL
Bone Marrow	Diffuse infiltration by CD68+ macrophages	Increased CD68 + macrophages
ECHO	EF 25%, severe left ventricular hypokinesis	EF 21%, global decrease in systolic function

Figure 1 The bone marrow biopsy shows hypercellularity with increased megakaryocytes (A, H&E x40). The immunohistochemical studies show increased interstitial macrophages by CD68 (PGM1) (B, x40); and they are type 2 macrophages negative for pSTAT1 (C, x 0) but positive for CD163 (D, x40).

Figure 2 The bone marrow biopsy shows hypocellularity with left-shifted myelopoiesis (A, H&E x 0). Immunohistochemical studies shows increased interstitial macrophages by CD68 (PGM1) (B, x40); they are type 2 macrophages (M2) negative for pSTAT1 (C, x 0) and positive for CD163 (D, x40).

Zhou JY, Martinez JA, Shen JP. Lamotrigine-induced hemophagocytic lymphohistiocytosis with Takotsubo cardiomyopathy: a case report. Journal of Medical Case Reports. 2019;13(1):345.

 Google Scholar

Otillio JK, Harris JK, Tuuri R. A 6-year-old girl with undiagnosed hemophagocytic lymphohistiocytosis and takotsubo cardiomyopathy: a case report and review of the literature. Pediatr Emerg Care. 2014;30(8):561–565.

 Web of Science ®Google Scholar

Takeoka Y, Nakamae M, Nakamae H, et al. Two cases of ampulla (takotsubo-shaped) cardiomyopathy associated with hemophagocytic lymphohistiocytosis. Acta Haematol. 2007;117(4):205–210.

 Web of Science ®Google Scholar

Prakash PB, Raj S. Cytokine induced myocardial dysfunction in a patient with hemophagocytic lymphohistiocytosis. J Am Coll Cardiol. 2016;67(13_Supplement):1061. doi:10.1016/S0735-1097(16)31062-2

 Web of Science ®Google Scholar

Abdin A. Takotsubo Cardiomyopathy Due to Hemophagocytic Lymphohistiocytosis. CHEST. 2016;150(4):435A.

 Web of Science ®Google Scholar

Ullah W, Hamid M, Grover H, Figueredo VM, Inayat F. Takotsubo cardiomyopathy secondary to haemophagocytic lymphohistiocytosis in HIV patients: a comprehensive review. BMJ Case Rep. 2019;12(4).

 Google Scholar

Chizinga M, Kalra SS, Innabi A, Rackauskas M, Ataya A, Emtiazjoo A. Macrophage activating syndrome causing decompensated right heart failure. Respir Med Case Rep. 2021;33:101409. doi:10.1016/j.rmcr.2021.101409

 Web of Science ®Google Scholar