Hemophagocytic Lymphohistiocytosis and Gastrointestinal Bleeding: What a Surgeon Should Know

Table 1

Author		Age	Concomitant systemic disease	Predominant symptoms and signs	Treatment; (surgical/medical)	Outcome
Kanaji et al. [10]	1	25	—	Fever, abdominal pain, and lower GI bleeding	Methylprednisolone (1000 mg/day for 3 consecutive days), g-globulin (0.5 g/day for 3 consecutive days), broad-spectrum antibiotics (PAPM 1 g/day), gabexate mesilate (2500 mg/day), ulinastatin (20 ×   U/day), and citicoline (1 g/day).	Survived
Eguchi [11]	1	44	Lupus nephritis	Melena, colonic perforation, and peritonitis	CMV immunoglobulin, methyl-prednisolone (1 g/day for three days), prednisolone (60 mg/day), and ganciclovir (dose not specified). Colonoscopic clipping and embolisation of a bleeding sigmoid ulcer	Died
Bhagwati et al. [12]	1	55	—	Fever, abdominal pain, GI bleeding, and acute renal failure	Prednisolone (30 mg/day) and amphotericin and ganciclovir (doses not specified).; Multiple transfusions	Died
Koketsu et al. [13]	1	35	—	Lower GI bleeding and fulminant “ulcerative colitis”	Methylprednisolone (1,000 mg/day for 3 consecutive days), subtotal colectomy, and ileostomy with rectal preservation	Survived
Nakase et al. [14]	2	68	—	Massive GI bleeding	https://www.jstage.jst.go.jp/browse/rinketsu/31/2/_contents?	Died
		80	—	Loss of consciousness and MOF	https://www.jstage.jst.go.jp/browse/rinketsu/31/2/_contents?	Died
Ina et al. [15]	1	76	—	Abdominal pain, massive GI bleeding, and jejunal ulcers	Jejunal resection (30 cm) after per operatory endoscopy	Survived
Yashima et al. [16]	1	63	Aplastic anemia	Massive GI bleeding and MOF	Methylprednisolone pulse therapy	Died
Wu et al. [17]	1	23	Hepatitis A and hepatitis C	Fever, jaundice, and massive GI bleeding	Bibliotheque	Died
Takai et al. [18]	1	20	—	Fever, hepatosplenomegaly, and massive GI bleeding	https://www.jstage.jst.go.jp/browse/rinketsu/31/2/_contents?	Died
Hayakawa et al. [19]	1	73	Polyarteritis nodosa	Massive GI bleeding	Methylprednisolone pulse therapy, intravenous immunoglobulin (5,000 mg for 3 days), and weekly intravenous VP-16 (200 mg/day). Embolisation of ileum artery branches	Died
N’Guyen et al. [20]	4	32.2 ± 3.7	Human; cytomegalovirus infection present in all 4 patients	Fever, cytopenia, hyperferritinemia, hypertriglyceridemia, and GI bleeding; one patient with pericarditis and tamponade, one with colitis, and one with 2 necrotic gastric ulcers	Corticoids and antiviral therapy (ganciclovir and/or valganciclovir) + anti-TNF	Survived
					Corticoids and antiviral therapy (ganciclovir and/or valganciclovir) + anti-TNF	Survived
					Corticoids and antiviral therapy (ganciclovir and/or valganciclovir) + total colectomy	Survived
					Corticoids and antiviral therapy (ganciclovir and/or valganciclovir). Emergency surgery for 2 gastric ulcer perforations (operation not specified)	Died
Tunç and Ayata [21]	1	4	Visceral leishmaniasis	High fever, malaise, fatigue, massive GI bleeding, and opportunistic infections	Immunoglobulin (IVIG) (400 mg/kg for 5 days), antibiotics (ceftazidime (100 mg/kg/24 h) and amikacin sulfate (15 mg/kg/24 h)), AmBisome (3 mg/kg/day for 5 days), and methylprednisolone (30 mg/kg/day) for 3 consecutive days	Died
Celkan et al. [22]	2	1.5	—	Submandibular lymphadenopathy and hepatomegaly massive lower GI bleeding	Immunoglobulin (0.5 g/kg), steroids (10 mg/m^2), etoposide (150 mg/m^2), cyclosporine (6 mg/kg), and recombinant; factor VIIa (rFVIIa) 90 μg/kg (total 4 mg). Resection of 12 cm of Ileum	Died
		4	—	Generalized edema and massive GI bleeding	Recombinant factor VIIa (rFVIIa) 90	Died

MOF: multiorgan failure.