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Unless the underlying cause is already apparent (and being appropriately managed), the presence of pancytopenia always warrants investigation by a haematologist, and the presence of severepancytopenia (symptomatic anaemia, WBC <0.5 x 10^9/L [<500/microlitre], and platelets <20 x 10^9/L [<20x10^3/microlitre]) calls for urgent investigation (within 24-48 hours).
Flow diagram forevaluation of pancytopenia. Abbreviations: PNH, paroxysmal nocturnal haemoglobinuria; IBMFS, inherited bone marrow failure syndromes
From the collection of JKD
A thorough history and physicalexamination are always required, preferably conducted by a haematologist. An FBC and examination of peripheral blood film by a haematologist are essential. Bone marrow examination by aspirate and biopsy isalmost always required as well.
History
The causes of pancytopenia are diverse, and likely causes of pancytopenia differ in children and adults. Particular attention must be paid to patient and familyhistory. Of significance is any history of previous pancytopenia, aplastic anaemia, inherited bone marrow failure syndromes (IBMFS), early fetal loss, history of cancer, metabolic disorders, liverdisease, or connective tissue disorders.
The most common cause of transient pancytopenia in all age groups is cytotoxic chemotherapy and radiotherapy. The symptoms and signs of pancytopenia relate to theblood cell lineages affected (RBCs, WBCs, and platelets). Mild pancytopenia is often symptomless and detected incidentally when an FBC is performed for another reason. Spontaneous mucosal bleeding(gums, GI tract), petechiae, and purpura with easy bruising secondary to thrombocytopenia are usually the first symptoms to develop directly related to more severe pancytopenia. This is often followedby symptomatic anaemia (fatigue, shortness of breath, dependent oedema, chest pain in patients with ischaemic disease) and bacterial infection secondary to neutropenia (fever, mucositis, abscesses,...