Breathless 2

Blood & Lymphoreticular System


Step 1: View Clinicals

A 12 year old girl presents with worsening exertional dyspnea, fatigue, and multiple episodes of fever over the last 3 weeks. She also noticed several bruises over her legs during the last week which could not be attributed to trauma.


She had an episode of glandular fever 2 months ago, with an uneventful recovery. Her medical history is otherwise unremarkable, and there is no family history of hematologic disorders.


She is not on any medications, and consumes a normal diet.


Step 2: Order Relevant Investigations

1. Full Blood Count

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WBC: 2,300/mm3 (4,000 - 11,000)

Neutrophils: 15% (50-70)

Lymphocytes: 80% (20-40)

RBC: 2.6 x 10^12/L (4.0-5.4 × 10^12)

Hb: 6.8 g/dL (11.5-13.5)

Plt: 17,000/mm3 (150,000 - 400,000)

Reticulocytes: 0.6% (0.2-2)

2. Blood Picture

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There is pancytopenia with macrocytosis, anisopoikilocytosis and small platelets. There are no dysplastic neutrophils, blast cells, or other abnormal cells.

3. Bone Marrow Aspiration, Biopsy and Cytogenetics

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The aspirate was insufficient for evaluation.


The bone marrow biopsy was markedly hypocellular (only 20% cellularity) with a profound reduction in haematopoietic cells, with the marrow being largely replaced by fat cells. No malignant infiltration or fibrosis was seen.


Cytogenetic examination revealed a normal karyotype. Flow cytometry with anti-CD55 and anti-CD59 antibodies excludes paroxysmal nocturnal hemoglobinuria clones. PCR failed to detect the Ebstein-Barr virus (EBV) genome in BM nucleated cells.

4. HLA Typing

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Identifies a sister with all 8 of 8 HLA matching.

Step 3: Select Appropriate Management

1. Transfuse Irradiated Blood Products

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2. Bone Marrow Transplantation

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3. Recombinant Erythropoietin

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4. Prophylactic Antibiotics

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