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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 Childrens Hospital at London


Health Science Center (LHSC) with ahistory 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 og 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 og 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

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 Castlemans disease of the hyaline vascular subtype.

Case

Ten weeks after surgery, his appetite had normalized and he had gained
7kg with increasing hemoglobin to 127 g/L

About Castlemans disease

Castlemans 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

-Symptoms
Fatigue
Anorexia
Rashes
Anemia
Trombocytopenia
Hepatosplenomegaly

Patophysiology
IL-6

Blocking Iron release from


Macrophages

Hepcidin

Inhibit Iron absorption in


Intestinal

Iron deficiency anemia refractory


to oral iron replacement therapy

Therapy

Surgical excision is the treatment of choice for unicentric disease

Radiation therapy

Prognosis

Unicentric disease carries an excellent prognosis with complete


resolution of symptoms in over 90% of cases following tretment

Conclusion

Castlemans 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.

Thank You

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