Celiac disease associated with aplastic anemia in a 6-year-old girl: a case report and review of the literature | Journal of Medical Case Reports

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A 6-year-old South Asian girl presented in October 2013 with generalized bruises, undocumented fever, and eczematous rashes of 1 month’s duration. She had had six or seven episodes of loose stools per day for 3 months accompanied by loss of appetite. She was a known asthmatic. Her family history was unknown as she was an adopted child. Her foster parents did not notice any food allergens. On examination, she was short with a height of 98 cm (<5th percentile) and weight of 13.5 kg (< 5th percentile). There was pallor, multiple generalized petechiae, and eczematous patches on upper back and behind her ears with typical Fanconi facies including microcephaly and microphthalmia. A systemic examination was normal and no lymphadenopathy was appreciated.

Investigations revealed hemoglobin of 9.0 g/dl, platelet count of 67 × 109/L, and mean corpuscular volume (MCV) and mean corpuscular hemoglobin (MCH) of 75.3 fl and 24.2 pg, respectively with no atypical cells. A liver function test (LFT), renal function test (RFT), urine examination, and coagulation profile were normal and blood culture was sterile. Ultrasonography of her abdomen and portal venous Doppler were normal. Antinuclear antibodies (ANA) titer was negative. Mitomycin C was used to detect chromosomal breakages to rule out Fanconi anemia and results were negative with 0.58 breaks/cell. Investigations for malabsorption revealed gamma A immunoglobulin (IgA) tissue transglutaminase levels to be 370 IU/ml (normal < 12 IU/ml), anti-gliadin antibodies to be 140 IU/ml (normal < 12 IU/ml), but normal thyroid profile. A jejunal biopsy showed complete villous atrophy with increased intraepithelial lymphocytes consistent with diagnosis of CD. She was managed with antibiotics, packed red cells, and platelet support and was put on a gluten-free diet. She was supplemented with multivitamins, vitamin C, and iron. She was discharged on day 17 of hospital stay with hemoglobin of 7.9 g/l, total leukocyte count of 2.5 × 109/l, and platelet count of 46 × 109/l. A bone marrow biopsy was advised. The household was noncompliant to follow ups with yearly follow ups showing varying platelet levels from 34 × 109/L to 124 × 109/L.

The child presented in October 2016 with nonproductive cough, fever, respiratory distress for 2 months, and a history of recurrent infections over the past year. Her hemoglobin was 7.5 g/l, total leukocyte count of 3.5 × 109/l, and platelet count of 80 × 109/l. High-resolution computed tomography (HRCT) was suggestive of a lung abscess. Lung culture revealed Pseudomonas aeruginosa. GeneXpert, galactomannan, and β-d-glucan (BDG) antibodies were negative. She was managed symptomatically with blood transfusions and platelet support. A bone marrow trephine biopsy was done after consent that revealed hypocellular bone marrow with decreased lymphoid cells, plasma cells, erythroid and myeloid precursors with occasional megakaryocytes. We advised that the child have a bone marrow transplantation but the family refused due to financial constraints. The parents were counseled about the nature of the disease and treatment modalities. She is managed with packed red cells, platelet support, and a gluten-free diet; she showed an increasing trend in platelet count on last follow up.

Table 

1

shows a timeline for our patient’s past medical history and follow-up visits as well as interventions.

Table 1

Timeline of patient’s medical history

October 2013

Presented with bruises, undocumented fever, and eczematous rashes for 1 month. Petechiae all over the body. Loose stools daily for 3 months with loss of appetite.

Hb 9.0 g/dl,

platelet 67 × 109/L,

MCV 75.3 fl,

MCH 24.2 pg.

Normal LFT, RFT, urine examination, and coagulation profile. Blood culture was sterile.

ANA titer was negative.

Chromosomal breakages was negative with 0.58 breaks/cell,

IgA tTG 370 IU/ml,

anti-gliadin antibodies 140 IU/ml.

Jejunal biopsy – complete villous atrophy with increased intraepithelial lymphocytes.

Antibiotics, packed red cells, and platelet support given.

Put on a gluten-free diet, supplemented with multivitamins, vitamin C, and iron.

She was discharged on day 17 of hospital stay with Hb 7.9 g/l,

TLC 2.5 × 109/l,

platelet count of 46 × 109/l, and gluten-free diet. Bone marrow biopsy was advised.

Noncompliant with yearly follow ups.

Varying platelet levels from 34 × 109/L to 124 × 109/L.

Gluten-free diet

October 2016

Nonproductive cough, fever, respiratory distress for 2 months.

History of recurrent infections over the year

Lung abscess and a cavitary lesion seen on HRCT.

Lung culture revealed Pseudomonas aeruginosa.

GeneXpert, galactomannan, and BDG antibodies were negative. A bone marrow trephine biopsy revealed hypocellular bone marrow.

She is planned for bone marrow transplantation.

Parents were counseled about the disease and treatment.

Managed with packed red cells, platelet support, and a gluten-free diet. She showed an increasing trend in platelet count.

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