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Intra-abdominal mass and iron

deficiency anemia in a 15-year-old


boy: Case report and literature review
Nalendra Tri W – 030.12.185 – Koas Interna
Case

 A 15-year-old boy was referred to the Children’s Hospital at London Health


Science Center (LHSC) with a history of severe iron deficiency anemia
unresponsive to oral iron replacement therapy.
 Two month previously he had presented to his community pediatrician
complaining of fatigue. He was diagnosed with iron deficiency anemia
and started on oral iron replacement therapy.
 He subsequently presented to his local emergency department with
testicular pain diagnosed as orchitis.
Case

 During assessment, bloodwork revealed a hypochromic microcytic anemia


with a hemoglobin of 68 g/L despite 2 month of oral iron therapy.
 At the referral, the patient reported significant failure to thrive with less than
2 kg of weight gained over the past 6 years and a current weight of only 29
kg.
 This condition accompanied by progressive fatigue, anorexia, pallor and
extertional dyspnea over the past year.
Case

 On Physical exam patient have chronic low grade fever. He was pale,
weight under 3rd percentile for age, with BMI of 14,9 and tanner stage 2
pubertal development.
 No lymphadenopathy or other anomalies noted.
Case

 Laboratory investigation revealed a hypochromic microcytic anemia with


hemoglobin of 68 g/L, White blood cell count 6.9/L, platelet 548/L. low
serum iron 3 umol/L. ESR 101mm/h and CRP 161mg/L
Case

 Consultation were made to hematology, oncology, endocrinology,


rheumatology, infectious disease, psychiatry, and gastroenterology.
 2 course of IV iron were administered with mild improvement in his
hemoglobin to 73g/L.
 Upper endoscopy and colonoscopy failed to identify any gastrointestinal
blood loss / other diseases.
 On MRE and capsule endoscopy to assess for small bowel lesions and
revealed a homogenous ovoid mestenteric mass.
Case
Case

 The patien was consented for excisional biopsy of this lesions.


 At laparotomy a discrete 4 cm lymphoid-appearing mass was found at the
base of mesentery.
 The mass was removed without complication and no other abnormalities
were found.
 Pathology revealed Castleman’s disease of the hyaline vascular subtype.
Case
Case

 Ten weeks after surgery, his appetite had normalized and he had gained
7kg with increasing hemoglobin to 127 g/L
About Castleman’s disease

 Castleman’s disease is a rare lymphoproliferative disease with a diverse


clinical spectrum
 Mediastinal involvement is the most common disease site but any lymph
node may be involved.
 10% cases are Intra-abdominal
Clinical Presentation

Unicentric Multicentric

Hyaline Vascular Plasmacytic / mixed cellularity

Asymptomatic β-Symptoms
Fatigue
Anorexia
Rashes
Anemia
Trombocytopenia
Hepatosplenomegaly
Patophysiology

IL-6 Hepcidin

Blocking Iron release from Inhibit Iron absorption in


Macrophages Intestinal

Iron deficiency anemia refractory


to oral iron replacement therapy
Therapy

 Surgical excision is the treatment of choice


 Radiation therapy
Conclusion

 Castleman’s disease is a rare lymphoproliperative disorder that can cause


a variety of insidious and progressive symptoms.

 Awareness of this disease can lead to earlier diagnosis and treatment


limiting associated morbidity.
Reference

 [1] Roca B. Castleman’s disease. A review. AIDS Rev 2009;11:3e7.


 [2] Dispenzieri A, Armitage JO, Loe MJ, Geyer SM, Allred J, Camoriano JK, et al. The clinical
spectrum of Castleman’s disease. Am J Hematol 2012;87: 997e1002.
 [3] Zhou N, Huang CW, Huang C, Liao W. The characterization and management of
Castleman’s disease. J Int Med Res 2012;40:1580e8.
 [4] Arlet JB, Hermine O, Darnige L, Ostland V, Westerman M, Badoual C, et al.
Irondeficiency anemia in Castleman disease: Implication of the interleukin6/hepcidin
pathway. Pediatrics 2010;126:e1608e12.
 [5] Galeotti C, Boucheron A, Guillaume S, Koné-Paut I. Sustained remission of multicentric
Castleman disease in children treated with tocilizumab, an antiinterleukin- 6 receptor
antibody. Mol Cancer Ther 2012;11:1623e6.
 [6] Song SN, Tomosugi N, Kawabata H, Ishikawa T, Nishikawa T, Yoshizaki K. Down
regulation of hepcidin resulting from long-term treatment with an anti-IL-6 receptor
antibody (tocilizumab) improves anemia of inflammation in multicentric Castleman
disease. Blood 2010;116:3627e34.
Thank You