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Paeds Vivashaematology-oncology-and-transfusion

Paeds Vivas · haematology-oncology-and-transfusion

Pancytopenia and marrow infiltration: Viva

Branching clinical structured oral on pancytopenia and marrow infiltration in children, covering the empty-versus-full marrow distinction, the leucoerythroblastic film and myelophthisis, the urgent diagnostic pathway from full blood count and film to bone marrow aspirate and trephine biopsy with flow cytometry and cytogenetics, the stabilisation with irradiated leucodepleted transfusion, tumour lysis prophylaxis with rasburicase and empiric antipseudomonal cover, the cause-specific definitive therapy for acute leukaemia, acquired and inherited marrow failure, Down syndrome transient myeloproliferative disorder, neuroblastoma, Langerhans cell histiocytosis and parvovirus B19 pure red cell aplasia, and the classic diagnostic pitfalls around the mediastinal mass and the evolving aplastic anaemia.

branching clinical structured oral5 min readVerification in progress

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RACP DWERACP DCEMRCPCH Clinical
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Target exams

RACP DWERACP DCEMRCPCH Clinical
Prompt
A four-year-old boy is brought in pale, covered in bruises, with a three-week history of fatigue and a fever. His full blood count shows haemoglobin 62 g per litre, neutrophils 0.4 times ten to the nine per litre, platelets 22 times ten to the nine per litre, and the peripheral film shows blasts. The examiner asks how you frame the problem, what you do in the first hours, how you confirm the diagnosis, and how you would counsel the family.

This is a branching oral built to probe the reasoning that holds the empty-versus-full marrow distinction at the centre, and to expose the candidate who has memorised the headline without the corners. The questions escalate from the framing to the stabilisation, the diagnosis, and the definitive management, with deliberate probes into the pitfalls. [3]

Opening question: framing the problem

The examiner opens with the film and the count and asks: how do you frame this problem in a single sentence, and what is your first priority? [1]

A strong answer names pancytopenia, the fall in all three lineages, and states that the first decision is whether the marrow is empty (failure) or full (infiltration). The circulating blasts with organomegaly settle the direction here: this is a marrow malignancy until proven otherwise. The first priority is the resuscitation of the unstable elements, because a child with a haemoglobin of 62, a neutrophil count of 0.4, and a fever is at risk of bleeding, infection, and the oncologic emergencies. [12]

Model answer. This child has pancytopenia with circulating blasts, which is acute leukaemia with marrow infiltration until proven otherwise. My first priority is to resuscitate the dangerous elements, the anaemia, the bleeding risk, the neutropenic fever, and the tumour lysis risk, and then to confirm the diagnosis with an urgent bone marrow aspirate and trephine biopsy in a specialist centre. [3]

Probe one: the resuscitation

The examiner presses: tell me exactly what you do in the first four hours, and why you choose those blood products. [12]

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References7ShowHide
  1. [1]Bhatnagar SK, Chandra J, Narayan S Pancytopenia in children: etiological profile J Trop Pediatr, 2005.PMID 16014764
  2. [3]Hunger SP, Mullighan CG Acute Lymphoblastic Leukemia in Children N Engl J Med, 2015.PMID 26465987
  3. [6]Sas V, Blag C, Zaharie G Transient leukemia of Down syndrome Crit Rev Clin Lab Sci, 2019.PMID 31043105
  4. [9]Means RT Jr Pure red cell aplasia Blood, 2016.PMID 27881371
  5. [10]Yoshida N Recent advances in the diagnosis and treatment of pediatric acquired aplastic anemia Int J Hematol, 2024.PMID 36867357
  6. [11]Janssens AM, Offner FC, Van Hove WZ Bone marrow necrosis Cancer, 2000.PMID 10760751
  7. [12]Prusakowski MK, Cannone D Pediatric Oncologic Emergencies Hematol Oncol Clin North Am, 2017.PMID 29078932
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