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Hemophagocytic Lymphohistiocytosis and Gastrointestinal Bleeding: What a Surgeon Should Know.

Popeskou S, Gavillet M, Demartines N, Christoforidis D - Case Rep Surg (2015)

Bottom Line: When intestinal bleeding is present, the management is very challenging with extremely high mortality rates.Early diagnosis and treatment seem to be the most important factors for a successful outcome.We present two cases and review another 18 from the literature.

View Article: PubMed Central - PubMed

Affiliation: Department of Surgery, Lugano Regional Hospital, Switzerland.

ABSTRACT
This paper presents to the surgical community an unusual and often ignored cause of gastrointestinal bleeding. Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH) is a rare medical entity characterized by phagocytosis of red blood cells, leucocytes, platelets, and their precursors in the bone marrow by activated macrophages. When intestinal bleeding is present, the management is very challenging with extremely high mortality rates. Early diagnosis and treatment seem to be the most important factors for a successful outcome. We present two cases and review another 18 from the literature.

No MeSH data available.


Related in: MedlinePlus

Hemophagocytosis in the bone marrow of Case 2.
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Related In: Results  -  Collection


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fig1: Hemophagocytosis in the bone marrow of Case 2.

Mentions: Two days after surgery, the patient became septic. A thoracoabdominal CT-scan showed bilateral pleural effusions but no specific septic source. In the absence of radiological signs that would explain the patient's septic status, a “second look” laparotomy was performed but no source of sepsis was identified. Subsequently, the patient's condition deteriorated rapidly. He developed disseminated intravascular coagulation and finally a new episode of GI bleeding. Based on the above listed observations together with ferritin levels at 15000 mcg/L, HLH was suspected and a treatment with CHOP regimen (cyclophosphamide 750 mg/m2 day 1, doxorubicin 50 mg/m2/day 1, Oncovin 1.4 mg/m2/day 1, and prednisone 60 mg/m2/day 1 to 5) plus etoposide was initiated. No bone marrow biopsies were performed as death due to hemodynamic instability followed the next day. Autopsy revealed a NK-T extranasal EBV + lymphoma with intestinal localization. Bone marrow presented with massive hemophagocytosis without any evidence of tumor infiltration (see Figure 1).


Hemophagocytic Lymphohistiocytosis and Gastrointestinal Bleeding: What a Surgeon Should Know.

Popeskou S, Gavillet M, Demartines N, Christoforidis D - Case Rep Surg (2015)

Hemophagocytosis in the bone marrow of Case 2.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4496477&req=5

fig1: Hemophagocytosis in the bone marrow of Case 2.
Mentions: Two days after surgery, the patient became septic. A thoracoabdominal CT-scan showed bilateral pleural effusions but no specific septic source. In the absence of radiological signs that would explain the patient's septic status, a “second look” laparotomy was performed but no source of sepsis was identified. Subsequently, the patient's condition deteriorated rapidly. He developed disseminated intravascular coagulation and finally a new episode of GI bleeding. Based on the above listed observations together with ferritin levels at 15000 mcg/L, HLH was suspected and a treatment with CHOP regimen (cyclophosphamide 750 mg/m2 day 1, doxorubicin 50 mg/m2/day 1, Oncovin 1.4 mg/m2/day 1, and prednisone 60 mg/m2/day 1 to 5) plus etoposide was initiated. No bone marrow biopsies were performed as death due to hemodynamic instability followed the next day. Autopsy revealed a NK-T extranasal EBV + lymphoma with intestinal localization. Bone marrow presented with massive hemophagocytosis without any evidence of tumor infiltration (see Figure 1).

Bottom Line: When intestinal bleeding is present, the management is very challenging with extremely high mortality rates.Early diagnosis and treatment seem to be the most important factors for a successful outcome.We present two cases and review another 18 from the literature.

View Article: PubMed Central - PubMed

Affiliation: Department of Surgery, Lugano Regional Hospital, Switzerland.

ABSTRACT
This paper presents to the surgical community an unusual and often ignored cause of gastrointestinal bleeding. Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH) is a rare medical entity characterized by phagocytosis of red blood cells, leucocytes, platelets, and their precursors in the bone marrow by activated macrophages. When intestinal bleeding is present, the management is very challenging with extremely high mortality rates. Early diagnosis and treatment seem to be the most important factors for a successful outcome. We present two cases and review another 18 from the literature.

No MeSH data available.


Related in: MedlinePlus