PNH causes a wide range of signs and symptoms
The name—paroxysmal nocturnal hemoglobinuria—literally means to have episodes of hemoglobin in the urine during the night. It was originally thought that the release of hemoglobin only occurred at night.
In reality, the destruction of the RBCs occurs constantly in PNH patients, a disease process known as chronic intravascular hemolysis. [1] Although hemoglobinuria is often assumed to be the main sign of PNH, only 26% of patients actually have this symptom when they are first diagnosed. [2] It was mistakenly thought that hemoglobinuria only occurred at night. [3]
As mentioned above, though hemoglobinuria may be more noticeable in the morning, RBC destruction, as well as the other signs and symptoms of PNH, may occur at any time of the day or night in a chronic fashion, punctuated by intense episodes, or paroxysms, of increased hemolysis/hemoglobinuria. [1,3]
Signs and symptoms of PNH include [1,3,4]:
- Anemia (requiring transfusions)
- Fatigue (can be disabling)
- Thrombosis
- Hemoglobinuria (dark urine)
- Abdominal pain
- Dyspnea (difficulty breathing)
- Dysphagia (difficulty swallowing)
- Erectile dysfunction
References: 1. Rosse WF, Hillmen P, Schreiber AD. Immune-mediated hemolytic anemia. Hematology (Am Soc Hematol Educ Program). January 2004:48-62.
2. Dacie JV, Lewis SM. Paroxysmal nocturnal hemoglobinuria: clinical manifestations, haematology, and nature of the disease. Ser Haematol.
1972;5:3-23. 3. Brodsky RA. Paroxysmal nocturnal hemoglobinuria. In: Hoffman R, Benz EJ, Shattil SJ, et al, eds. Hematology. 4th ed. Philadelphia, Pa: Elsevier Churchill Livingstone; 2005:419-427. 4. Parker C, Omine M, Richards S, et al, for the International PNH Interest Group. Diagnosis and management of paroxysmal nocturnal hemoglobinuria. Blood. 2005;106:3699-3709.
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