Infectious Mimicry Complicates Diagnosis in Hemophagocytic Syndrome Caused by Anaplastic Large-Cell Lymphoma

Table 1

Comparison of the present case with previously published cases of ALCL-associated hemophagocytic syndrome.

	Present case	Krenova et al. [11]	Sovinz et al. [12]	Sevilla et al. [13]		Cho et al. [14]
Age, gender	20 years, M	17 years, F	15 years, M	16 years, F	5 years, F	25 years, M
History and physical exam	FUO, facial swelling, LAD, and uveitis	FUO, LAD, and splenomegaly	FUO, neck swelling, and splenomegaly	FUO, splenomegaly	FUO, abdominal pain, and headache	FUO, myalgia, weakness, jaundice, LAD, and splenomegaly
Labs
WBC (cells/L)	2.0 × 10^9	—	5.0 × 10^9	1.4 × 10^9	5.2 × 10^9	1.6 × 10^9
ANC (cells/L)	0.7 × 10^9	0.4 × 10^9	—	—	—	—
Hemoglobin (g/dL)	9.5	4.4	10.1	7	8.9	8.8
Platelets (cells/L)	145 × 10^3	46 × 10^3	167 × 10^3	84 × 10^3	213 × 10^3	88 × 10^9
Triglycerides (mg/dL)	284	316	218	—	—	113
Fibrinogen (mg/dL)	246	70	210	320	91	150
Ferritin (ng/mL)	13500	20 (g/L)	9270	5166	—	2240
Imaging studies	Soft tissue swelling, splenomegaly, fat stranding, and lung nodules	—	Cervical LAD	Mediastinal LAD, splenomegaly, and lung nodules	Splenomegaly, mediastinal and hilar LAD	Cervical, mediastinal, and abdominal LAD, hepatomegaly, and splenomegaly
Initial diagnosis	IAHS	Hepatitis	IAHS	IAHS	IAHS	ALCL
Biopsy
Lymph node	—	ALCL	HPS; ALCL	ALCL	ALCL	ALCL
Soft tissue	Necrosis, HPS	—	—	—	—
Bone marrow	HPS	HPS; ALCL	HPS	HPS	HPS	HPS
Lung nodule	Nonspecific	—	—	ALCL	—	—
Final diagnosis	ALCL	ALCL	ALCL	ALCL	ALCL	ALCL

Abbreviations: M: male; F: female; FUO: fever of unknown origin; LAD: lymphadenopathy; WBC: white blood cells; ANC: absolute neutrophil count; ALCL: anaplastic large cell lymphoma; HPS: hemophagocytic syndrome; IAHS: infection-associated hemophagocytic syndrome.