Maria Arini Lopez, PT, DPT, CSCS, CMTPT, CIMT is a freelance medical writer and Doctor of Physical Therapy from Maryland. She has expertise in the therapeutic areas of orthopedics, neurology, chronic pain, gastrointestinal dysfunctions, and rare diseases especially Ehlers Danlos Syndrome.
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, chronic hematologic disorder in which the premature destruction of erythrocytes results in the widespread circulation of free hemoglobin in the blood and later excretion in the urine.1
Patients with PNH are not required to maintain any specific diet.2 However, some sources state that patients with PNH should follow the dietary and nutritional recommendations for patients with aplastic anemia, a condition that often accompanies PNH.3
Read more about PNH comorbidities
Individuals with aplastic anemia — and by extension, patients with PNH — are advised to maintain a healthy body weight and eat a diet that focuses on fresh, whole foods, such as fruits, vegetables, whole grains, and legumes. Patients should avoid fast food and highly processed, starchy, or heavily salted food.3
Salt increases water retention and may aggravate symptoms of pulmonary hypertension.4 Free hemoglobin results in a depletion of nitric oxide, which impairs vascular endothelial function. Pulmonary hypertension may be a complication of PNH.5,6
Diets rich in fruits and vegetables, such as Mediterranean, vegan, and vegetarian diets, are known to lower serum markers of hemostasis and inflammation and reduce the risk for venous thromboembolism (VTE). VTE is a major complication of PNH and the leading cause of death among patients with this disease. Consuming increased amounts of fruits and vegetables and reducing meat intake may help reduce the risk for VTE.7
Other sources recommend a neutropenic diet, especially for patients with PNH who have low white blood cell counts and are therefore prone to bacterial and fungal infections. A neutropenic diet avoids foods and situations conducive to infection (food poisoning), such as2,8:
- Buffets, salad bars, and crowded restaurants
- Raw meat, fish, nuts, and eggs
- Partially cooked eggs
- Unpasteurized or fermented beverages
- Aged cheeses or other food items
- Well water
Sugary and caffeinated beverages should be avoided, as water is the better choice and caffeinated drinks act as a diuretic. Patients with PNH should drink 8 glasses of water daily.8
Read more about PNH complications
Folate or folic acid supplementation promotes erythropoiesis in the bone marrow and may help compensate for the hemolytic anemia associated with PNH.9
Iron supplementation is recommended, especially for patients with PNH who have an iron deficiency. Iron deficiency in PNH may be due to intravascular hemolysis, which causes a loss of hemoglobin and its metabolic byproduct, iron-containing heme, through the urine.9
Read more about PNH treatment
Study on Supplementation for PNH
A 2020 study was conducted in China on the efficacy and safety of iron supplementation in patients with PNH and iron deficiency. Results showed increased hemoglobin levels in 20 of 36 patients (56%) who received oral iron supplementation and increased hemoglobin levels in 16 of 18 patients (89%) who received intravenous iron supplementation. Of the 36 patients who received oral iron supplementation, 6 did not respond well and received intravenous iron supplementation instead. The hemoglobin levels of 5 of these 6 patients improved following the switch.10
Read more about PNH clinical trials
Tips for Food Preparation
When working in the kitchen and preparing food, it’s important for patients with PNH to reduce the likelihood of food contamination and subsequent infection. Tips for these tasks include8:
- Maintain clean kitchen surfaces and utensils.
- Wash hands frequently before, during, and after food preparation.
- Wash or peel all fruits and vegetables before preparation or consumption.
- Use 2 separate cutting boards: one for meats and the other for fruits and vegetables. Bleach the meat cutting board after each use to minimize likelihood of bacterial contamination.
- Always cook meats to the recommended safe temperatures.
- Verify “sell-by” and “use-by” dates.
- Never leave food out on a counter or table for more than 2 hours.
- Dispose of leftovers within 48 hours.
- Dispose of any foods that look or smell strange. Never taste them.
- Dispose of eggs with cracked shells.
- Change hand towels daily and use disposable sponges or dish clothes to wash dishes by hand, or use the dishwasher.
Read more about PNH risk factors
- Paroxysmal nocturnal hemoglobinuria. Medline Plus. Accessed November 19, 2022.
- Is there a special diet I should be on that would help me feel better? AA MDS International Foundation. Accessed November 19, 2022.
- Paroxysmal nocturnal hemoglobinuria: what to know. PicnicHealth. Accessed November 19, 2022.
- Controlling salt and sodium consumption. Pulmonary Hypertension Association. Accessed November 19, 2022.
- Haw A, Palevsky HI. Pulmonary hypertension in chronic hemolytic anemias: pathophysiology and treatment. Respir Med. 2018;137:191-200. doi:10.1016/j.rmed.2018.02.020
- Shah N, Bhatt H. Paroxysmal nocturnal hemoglobinuria. StatPearls [Internet]. Updated August 1, 2022. Accessed November 19, 2022.
- Cundiff DK, Agutter PS, Malone PC, Pezzullo JC. Diet as prophylaxis and treatment for venous thromboembolism? Theor Biol Med Model. 2010;7:31. doi:10.1186/1742-4682-7-31
- Nutrition. AA MDS International Foundation. Accessed November 19, 2022.
- Paroxysmal nocturnal hemoglobinuria. NORD. Accessed November 19, 2022.
- Peng GX, Zhang L, Yang WR, et al. [Evaluation of the efficacy and safety of iron therapy in patients with paroxysmal nocturnal hemoglobinuria complicated with iron deficiency anemia]. [Article in Chinese]. Zhonghua Xue Ye Xue Za Zhi. 2020;41(8):671-674. doi:10.3760/cma.j.issn.0253-2727.2020.08.010
Reviewed by Kyle Habet, MD, on 11/30/2022.