Pancytopenia and marrow infiltration

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Target exams

Red flags

• A pale, bruising, febrile child with hepatosplenomegaly or lymphadenopathy has marrow malignancy until the bone marrow proves otherwise
• A leucoerythroblastic film with nucleated red cells and teardrop poikilocytes signals marrow infiltration, fibrosis or myelophthisis and demands urgent marrow examination
• A high blast count with a white cell count over one hundred times ten to the nine per litre risks leukostasis in the brain and lungs and needs urgent cytoreduction
• A mediastinal mass with stridor, superior vena cava obstruction or respiratory distress is an anaesthetic catastrophe waiting to happen; avoid sedation before securing the airway
• Severe acquired aplastic anaemia is defined by a neutrophil count under zero point five, platelets under twenty, and a corrected reticulocyte count under one percent, and carries a high early mortality without rapid treatment
• A sudden fall in haemoglobin with a reticulocyte count near zero in a child with a chronic haemolysis is parvovirus B19 transient pure red cell aplasia until excluded

Life stages

Care settings

Clinical exam formats

Board mappings

• Defines pancytopenia as a reduction in all three blood cell lineages and separates reduced marrow production from peripheral consumption or sequestration
• Names the common causes of marrow infiltration in a child: acute leukaemia, neuroblastoma, Langerhans cell histiocytosis, rhabdomyosarcoma and disseminated tuberculosis
• Recognises the red-flag pattern of a pale, bruising, febrile child with hepatosplenomegaly and lymphadenopathy as probable marrow malignancy needing urgent investigation
• Explains how marrow infiltration by blasts, tumour cells or fibrosis produces trilineage failure and a leucoerythroblastic film with nucleated red cells and teardrop poikilocytes
• Coordinates the urgent diagnostic pathway from full blood count and film through bone marrow aspirate, trephine biopsy, flow cytometry, cytogenetics and molecular testing to the named diagnosis
• Distinguishes acquired aplastic anaemia, inherited bone marrow failure syndromes, parvovirus B19 transient red cell aplasia, and Down syndrome transient myeloproliferative disorder from true leukaemia
• Pancytopenia is a reduction in haemoglobin, neutrophils under one point five times ten to the nine per litre, and platelets under one hundred and fifty times ten to the nine per litre
• Acute tumour lysis syndrome prevention for a high-risk marrow malignancy uses hyperhydration, allopurinol or rasburicase, and close biochemical monitoring
• Febrile neutropenia in a child on treatment for leukaemia is treated empirically with an antipseudomonal beta-lactam within one hour
• Counsels the family of a child newly diagnosed with acute leukaemia presenting as pancytopenia, explaining the diagnosis, the treatment phases and the prognosis
• Builds the multidisciplinary plan that runs paediatric haematology-oncology, infectious diseases, the diagnostic laboratory, the family and the primary care team together
• Synthesises the long-case child with inherited bone marrow failure into a surveillance and transition plan covering malignancy risk, transfusion, iron overload and stem cell transplant
• Level 1: Recognition of pancytopenia on a full blood count and identification of the red-flag features that mandate urgent referral
• Level 2: Application of the bone marrow aspirate and trephine biopsy pathway with flow cytometry and cytogenetics to reach a named diagnosis
• Level 3: Coordination of definitive therapy for marrow failure and malignancy, including transfusion support, transplant referral and long-term surveillance
• The definition of pancytopenia and the three pathogenetic mechanisms of reduced production, marrow replacement and peripheral consumption
• The leucoerythroblastic blood film and its association with marrow infiltration, fibrosis and myelophthisis
• The causes of marrow failure in childhood including aplastic anaemia, Fanconi anaemia and parvovirus B19 transient red cell aplasia
• Structured assessment of the pale, bruising child for pallor, petechiae, organomegaly, lymphadenopathy and signs of inherited marrow failure
• Communication of a new diagnosis of acute leukaemia or marrow failure to a child and family with appropriate honesty and support
• Recognition of the oncologic emergencies of hyperleukocytosis, tumour lysis syndrome and febrile neutropenia
• Pancytopenia in a child as a presentation that demands rapid separation of benign transient causes from marrow malignancy and failure
• The diagnostic roles of the peripheral blood film, bone marrow aspirate, trephine biopsy, flow cytometry and cytogenetics
• Down syndrome as a predisposition to transient myeloproliferative disorder and acute megakaryoblastic leukaemia
• Recognition of the red-flag clinical and laboratory features of pancytopenia that mandate urgent referral to paediatric haematology-oncology
• Application of the initial stabilisation of a child with severe pancytopenia including transfusion and sepsis precaution
• Coordination of the diagnostic and multidisciplinary pathway for a child with marrow infiltration or failure
• Canadian approach to the urgent evaluation and referral of a child with newly identified pancytopenia
• Bone marrow aspirate and trephine biopsy with ancillary studies as the diagnostic standard for marrow infiltration
• Inherited bone marrow failure syndromes and their surveillance for clonal evolution and malignancy