A 67-year-old female presents to the Emergency Department as a “code stroke”. Initial CT head confirms a large intracranial hemorrhage. Her CBC is shown below.
- What key investigations would you order for pancytopenia?
- How would you manage this patient?
Click for answer:
Diagnosis: aplastic anemia (AA)
CBC Clues:
(1) Pancytopenia with low reticulocyte count
(2) Absence of immature/blast cells
Q1: What key investigations would you order for pancytopenia?
Reticulocyte count, smear, Vit B12, iron studies
Q2: How would you manage this patient?
Supportive transfusions, target plt >100 given neurologic bleed, hematology referral
Secondary Causes for AA
• Drugs – benzene, chemotherapy, NSAID, carbamazepine
• Infections – EBV, CMV, parvovirus
• Autoimmune – SLE
• Miscellaneous – paroxysmal nocturnal hemoglobinuria
AA is a hematologic disorder characterized by two or more cytopenias. Patients will present with significant anemia (reticulocyte < 20), thrombocytopenia (< 20), and/or neutropenia (< 0.5). It can be primary or secondary (see Box for select secondary causes). In severe cases, this can lead to death due to risk of infection and hemorrhage. The mechanism is related to cytotoxic T-cell mediated destruction of hematopoietic stem cells, leading to reduced blood cell production. This is confirmed on bone marrow examination, which will demonstrate hypocellular marrow.
Important investigations include reticulocyte count, nutrition studies (Vitamin B12, iron studies), thorough pancytopenia work-up, and hemolysis work-up. A bone marrow biopsy is essential to rule out malignancy and confirm the diagnosis.
CBC Pearls
• Aplastic anemia is subacute pancytopenia
• Consider AA with low reticulocyte count and no immature cells/blasts
• O+ blood products can be given for urgent transfusion
Supportive blood transfusions to prevent severe anemia, hemorrhage, and infection are essential in aplastic anemia. Any signs of neurologic bleeding should prompt platelet transfusion to a target of > 100. The mainstay of therapy includes hematology referral for immunosuppression and hematopoietic stem cell transplant.