Langerhans cell histiocytosis with digestive tract involvement

Yadav, Satya Prakash; Kharya, Gaurav; Mohan, Neelam; Sehgal, A. D.; Bhat, Sunil; Jain, Sandeep; Kapoor, Gauri; Anupam, Sachdeva

doi:10.1002/pbc.22663

Cited by 46 publications

(35 citation statements)

References 15 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…This term can cause confusion as multiple organs may be severely affected at the moment of diagnosing LCH or during follow up. [1][2][3] It seems that the morphologic characteristics of the skin lesions, even their spontaneous regression, do not indicate the extent and prognosis of the disease.…”

Section: Discussionmentioning

confidence: 99%

Digestive Tract Symptoms in Congenital Langerhans Cell Histiocytosis

Vetter-Laracy

Salinas²,

Martín‐Santiago³

et al. 2014

Journal of Pediatric Hematology/Oncology

View full text Add to dashboard Cite

show abstract

Section: Discussionmentioning

confidence: 99%

Digestive Tract Symptoms in Congenital Langerhans Cell Histiocytosis

Vetter-Laracy

Salinas²,

Martín‐Santiago³

et al. 2014

Journal of Pediatric Hematology/Oncology

View full text Add to dashboard Cite

show abstract

“…High risk organs include hematopoietic cells, liver, spleen, and lungs; involvement of these organs portends a worse prognosis. 4,[8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25] Children with LCH of the bowel present early in life with a mean (±standard deviation) age at diagnosis of 8.6 (±8.2) months ( Table 1). 6,7 We reviewed the clinical data of 36 children with LCH of the bowel identified in the English literature.…”

Section: Discussionmentioning

confidence: 99%

Protein-Losing Enteropathy due to Intestinal and Colonic Involvement With Langerhans Cell Histiocytosis and Review of the Literature

Miller

Iyer

et al. 2013

Clin Pediatr (Phila)

View full text Add to dashboard Cite

A 4-month-old white female presented with a 2-month history of a diffuse petechial rash, intermittent fever, and worsening anemia and thrombocytopenia. She had a life-long history of spitting-up attributed to gastroesophageal reflux. However, over the preceding 2 weeks she developed nonbloody vomiting and a decrease in oral intake. During this time, she also developed diarrhea, having 5 bowel movements per day that contained mucus but no visible blood. She was treated sequentially with an H2-receptor antagonist and a proton-pump inhibitor without improvement. The patient was admitted to the hospital for further evaluation and treatment with a presumptive diagnosis of Langerhans cell histiocytosis (LCH). Examination revealed weight at the 10th percentile, length at the 25th percentile, head circumference at the 50th percentile, and weight-to-length ratio at the 25th percentile. There was no sclera icterus. Heart and lung examinations were normal. The abdomen was soft and nontender with hepatomegaly; the liver was palpable below the right costal margin and extended across the midline. No spleen was palpable. No lymphadenopathy was noted. Skin examination revealed a diffuse papular and petechial rash. Edema was present in the face, hands, and feet. The stool was positive for fecal occult blood.Pertinent laboratory studies included white blood cell count (8210/mm 3 ), hemoglobin (9.7 g/dL), hematocrit (30.5%), platelet count (49 000/mm 3 ), total protein (3.3 g/dL), and albumin (1.9 g/dL). A liver panel, serum electrolytes, and glucose were normal. Routine urinalysis was negative for proteinuria. Fecal α-1-antitrypsin level was elevated at 340 mg/dL (normal <55 mg/dL).Imaging studies did not reveal any bone lesions. A bone marrow aspirate and biopsy were performed and found to be normal. A punch biopsy of a skin lesion obtained at the time of the bone marrow procedure revealed LCH. When there was no improvement in gastrointestinal symptoms an upper endoscopy and colonoscopy were performed. Esophagogastroduodenoscopy (EGD) showed abnormal duodenal mucosa with edema, erythema, and a granular appearance with an overlying exudate (Figure 1). Colonoscopy revealed loss of vascular marking, consistent with edema. Duodenal biopsies showed focal mucosal erosion and a mononuclear cell infiltrate in the lamina propria consistent with LCH ( Figure 2A). The cells were positive by immunostaining for CD1a, supporting a diagnosis of LCH ( Figure 2B). Colonic biopsies also contained a mononuclear cell infiltrate and CD1a positive staining macrophages ( Figure 2C and D).Chemotherapy was initiated using the LCH-III protocol, which included vinblastine and prednisone. The total duration of the therapy was for 12 months. Vinblastine was given intravenously weekly for 12 weeks and then every 3 weeks for a total of 52 weeks with very few delays and without any major complications. Prednisone was given daily for 6 weeks and then weekly 3-day pulses orally for an additional 10 weeks. Thereafter, Velban was given every 3 weeks with 5 days of ...

show abstract

“…The clinical picture can vary but most often consists of bloody diarrhoea, non bloody diarrhoea or constipation, protein losing enteropathy, hypoproteinaemia and anaemia 4 5. It almost exclusively affects children below two years of age and it has been noted that over half of patients with LCH and GI involvement die within one and a half years of diagnosis 4 5. Here we describe the case of a young female who required combination chemotherapy to control her GI disease.…”

mentioning

confidence: 97%

“…While single system disease has a good prognosis, multisystem disease often requires intensive combination chemotherapy 2 3. Gastrointestinal (GI) involvement in LCH remains rare4 and its presence alone does not fit criteria for such treatment 3. The clinical picture can vary but most often consists of bloody diarrhoea, non bloody diarrhoea or constipation, protein losing enteropathy, hypoproteinaemia and anaemia 4 5.…”

mentioning

confidence: 99%

“…Gastrointestinal (GI) involvement in LCH remains rare4 and its presence alone does not fit criteria for such treatment 3. The clinical picture can vary but most often consists of bloody diarrhoea, non bloody diarrhoea or constipation, protein losing enteropathy, hypoproteinaemia and anaemia 4 5. It almost exclusively affects children below two years of age and it has been noted that over half of patients with LCH and GI involvement die within one and a half years of diagnosis 4 5.…”

mentioning

confidence: 99%

See 1 more Smart Citation

Gastrointestinal involvement in langerhans cell histiocytosis: consider treatment as for multi-system disease

Williamson

O'Neil²,

Walker³

et al. 2012

Arch Dis Child

View full text Add to dashboard Cite

Aims Langerhans cell histiocytosis (LCH) is a rare disorder characterised by abnormal proliferation of the Langerhans cell.1 While single system disease has a good prognosis, multisystem disease often requires intensive combination chemotherapy.2 3 Gastrointestinal (GI) involvement in LCH remains rare4 and its presence alone does not fit criteria for such treatment.3 The clinical picture can vary but most often consists of bloody diarrhoea, non bloody diarrhoea or constipation, protein losing enteropathy, hypoproteinaemia and anaemia.4 5 It almost exclusively affects children below two years of age and it has been noted that over half of patients with LCH and GI involvement die within one and a half years of diagnosis.4 5 Here we describe the case of a young female who required combination chemotherapy to control her GI disease. Methods A six month old female infant initially presented with a non-blanching erythematous rash involving the abdomen and groin, later developing bloody diarrhoea, intermittent bilious vomiting and failure to thrive. She was pale, wasted and developmentally delayed with a painless cystic lump on the occiput. There was lymphadenopathy and an enlarged spleen. Investigations revealed anaemia, thrombocytosis, elevated fibrinogen, a raised ESR and hypoalbuminaemia. Florid histiocytic inflammation of the small bowel was identified at endoscopy and a barium swallow showed narrowing of the distal duodenum and proximal jejunum without obstruction. Gut biopsies and skin samples confirmed the diagnosis of LCH. Results Dual therapy consisting of vinblastine and prednisolone was commenced with quick regression of her bone and skin disease. The addition of mercaptopurine and methotrexate was eventually required to control her GI disease. Almost three years post diagnosis she remains on this quadruple regimen as maintenance therapy. Her current disease status is that of regressing disease although her symptoms return towards the end of treatment intervals. Discussion The prevalence of GI involvement may be underestimated given its presentation; mild cases could be overlooked without endoscopic examination and biopsies. We may see a rise in recorded cases with increased availability of endoscopy. From our experience of this case we recommend that combination therapy is considered for cases of LCH with GI involvement.

show abstract

Langerhans cell histiocytosis with digestive tract involvement

Cited by 46 publications

References 15 publications

Digestive Tract Symptoms in Congenital Langerhans Cell Histiocytosis

Digestive Tract Symptoms in Congenital Langerhans Cell Histiocytosis

Protein-Losing Enteropathy due to Intestinal and Colonic Involvement With Langerhans Cell Histiocytosis and Review of the Literature

Gastrointestinal involvement in langerhans cell histiocytosis: consider treatment as for multi-system disease

Contact Info

Product

Resources

About