Paroxysmal Nocturnal Hemoglobinuria What every physician needs to know: Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal hematopoietic stem cell disease that can present with bone marrow failure, hemolytic anemia, smooth muscle dystonias, and thrombosis. The disease originates from a multipotent hematopoietic stem cell that acquires a mutation of the PIG-Agene. Expansion and differentiation of the…
Pancytopenia What every physician needs to know: A reduction in red cells, white cells, and platelets is referred to as pancytopenia. Pancytopenia usually reflects the bone marrow’s response to non-hematologic conditions (for example, infection, drugs, inflammation, malignancy, nutritional changes). It is important to rule out these conditions before considering much less common hematological diseases. The…
What every physician needs to know: Background Aplastic anemia (AA) can be inherited or acquired. Inherited forms usually present during the first decade of life, but in rare cases may manifest in adulthood. These two forms should be distinguished early on in a patient’s course. Acquired aplastic anemia, the subject of this chapter, is a…
Paroxysmal Nocturnal Hemoglobinuria What every physician needs to know: Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal hematopoietic stem cell disease that can present with bone marrow failure, hemolytic anemia, smooth muscle dystonias, and thrombosis. The disease originates from a multipotent hematopoietic stem cell that acquires a mutation of the PIG-Agene. Expansion and differentiation of the…
