Myelodysplastic syndrome
Background
- Acquired clonal disorder of hematopoietic stem cells, leading to hypercellular marrow, abnormal cell morphology, and cytopenias
- Idiopathic or secondary to chemotherapy or radiation
- May evolve into acute myeloid leukemia
Clinical Features
- Asymptomatic
- Signs/symptoms of anemia
- Signs/symptoms of infection if neutropenic
- Signs/symptoms of thrombocytopenia
- Splenomegaly
Differential Diagnosis
- Acute leukemia, infiltrative malignancy (e.g. lymphoma, multiple myeloma, metastatic carcinoma)
- Myelodysplastic syndrome, myelofibrosis
- Aplastic anemia
- Paroxysmal nocturnal hemoglobinuria
- Medication/radiation induced
- Tuberculosis, AIDS
- Leishmaniasis, brucellosis, histoplasmosis.
- Viruses causing aplastic anemia (hepatitis, Epstein-Barr virus, HIV, parvovirus B19)
- Systemic lupus erythematosus
- Hypersplenism
Evaluation
- CBC
- Marked normo or macrocytic anemia
- Leukopenia, commonly with abnormal neutrophil morphology
- Thrombocytopenia
- Peripheral smear
- Type and screen
- Infectious workup, if signs of infection present
- Evaluate for other causes of symptoms
Management
- See anemia, thrombocytopenia, neutropenic fever
- Outside of ED, treatments may include
- EPO, filgrastim, romiplostim, eltrombopag
- Lenalidomide
- Stem cell transplant