Abstract
We describe presenting features, treatment strategies, and follow-up events involving 41 patients (median age 39 years, range 1–81; 54% males) with high oxygen affinity (HOA) hemoglobinopathy-associated erythrocytosis, seen at our institution (1973–2020). Thirty-four (83%) patients carried β-chain (13 Malmo, 4 Olympia, 3 San Diego, 2 Wood) and 7 (17%) α-chain (4 Dallas and one each Columbia-Missouri, Jackson, and Wayne) variants. Median (range) hemoglobin (Hgb)/hematocrit (Hct), serum erythropoietin and p50 were 18 g/dL/52.9% (16–21.9/48–66), 10.4 mIU (4–36.3), and 20 mmHg (12–25), respectively. Family history was documented in 24 patients and history of thrombosis in two (5%). Treatment included phlebotomy in 23 and antiplatelet therapy in 21 patients. At a median follow-up of 10 years, 23 (56%) patients reported one or more symptoms that were thought to be related to their increased Hct while thrombosis was documented in 10 (24%) patients. Neither Hgb/Hct level nor active phlebotomy showed a significant correlation with either thrombotic or nonthrombotic symptoms (p >.1 in all instances). Among 23 pregnancies recorded, 78% resulted in live births and no fetal loss was attributed to erythrocytosis. The current study does not implicate Hgb/Hct level as a major contributor of morbidity in HOA hemoglobinopathy-associated erythrocytosis and suggests limited therapeutic value for phlebotomy.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 1647-1654 |
| Number of pages | 8 |
| Journal | American journal of hematology |
| Volume | 96 |
| Issue number | 12 |
| DOIs | |
| State | Published - Dec 1 2021 |
ASJC Scopus subject areas
- Hematology
Access to Document
Other files and links
Link to the citations in Scopus
(Video) (Webinar) Red Blood Cell Membrane Disorders: from diagnosis to clinical management
Fingerprint
Dive into the research topics of 'High-oxygen-affinity hemoglobinopathy-associated erythrocytosis: Clinical outcomes and impact of therapy in 41 cases'. Together they form a unique fingerprint.
View full fingerprint
Cite this
- APA
- Standard
- Harvard
- Vancouver
- Author
- BIBTEX
- RIS
Gangat, N., Oliveira, J. L., Hoyer, J. D., Patnaik, M. M., Pardanani, A. (2021). High-oxygen-affinity hemoglobinopathy-associated erythrocytosis: Clinical outcomes and impact of therapy in 41 cases. American journal of hematology, 96(12), 1647-1654. https://doi.org/10.1002/ajh.26375
High-oxygen-affinity hemoglobinopathy-associated erythrocytosis: Clinical outcomes and impact of therapy in 41 cases. / Gangat, Naseema; Oliveira, Jennifer L.; Hoyer, James D. et al.
In: American journal of hematology, Vol. 96, No. 12, 01.12.2021, p. 1647-1654.
Research output: Contribution to journal › Article › peer-review
Gangat, N, Oliveira, JL, Hoyer, JD, Patnaik, MM, Pardanani, A 2021, 'High-oxygen-affinity hemoglobinopathy-associated erythrocytosis: Clinical outcomes and impact of therapy in 41 cases', American journal of hematology, vol. 96, no. 12, pp. 1647-1654. https://doi.org/10.1002/ajh.26375
Gangat N, Oliveira JL, Hoyer JD, Patnaik MM, Pardanani A, Tefferi A. High-oxygen-affinity hemoglobinopathy-associated erythrocytosis: Clinical outcomes and impact of therapy in 41 cases. American journal of hematology. 2021 Dec 1;96(12):1647-1654. doi: 10.1002/ajh.26375
Gangat, Naseema ; Oliveira, Jennifer L. ; Hoyer, James D. et al. / High-oxygen-affinity hemoglobinopathy-associated erythrocytosis : Clinical outcomes and impact of therapy in 41 cases. In: American journal of hematology. 2021 ; Vol. 96, No. 12. pp. 1647-1654.
@article{ea3879a3e1844801b229392cc03c361f,
title = "High-oxygen-affinity hemoglobinopathy-associated erythrocytosis: Clinical outcomes and impact of therapy in 41 cases",
abstract = "We describe presenting features, treatment strategies, and follow-up events involving 41 patients (median age 39 years, range 1–81; 54% males) with high oxygen affinity (HOA) hemoglobinopathy-associated erythrocytosis, seen at our institution (1973–2020). Thirty-four (83%) patients carried β-chain (13 Malmo, 4 Olympia, 3 San Diego, 2 Wood) and 7 (17%) α-chain (4 Dallas and one each Columbia-Missouri, Jackson, and Wayne) variants. Median (range) hemoglobin (Hgb)/hematocrit (Hct), serum erythropoietin and p50 were 18 g/dL/52.9% (16–21.9/48–66), 10.4 mIU (4–36.3), and 20 mmHg (12–25), respectively. Family history was documented in 24 patients and history of thrombosis in two (5%). Treatment included phlebotomy in 23 and antiplatelet therapy in 21 patients. At a median follow-up of 10 years, 23 (56%) patients reported one or more symptoms that were thought to be related to their increased Hct while thrombosis was documented in 10 (24%) patients. Neither Hgb/Hct level nor active phlebotomy showed a significant correlation with either thrombotic or nonthrombotic symptoms (p >.1 in all instances). Among 23 pregnancies recorded, 78% resulted in live births and no fetal loss was attributed to erythrocytosis. The current study does not implicate Hgb/Hct level as a major contributor of morbidity in HOA hemoglobinopathy-associated erythrocytosis and suggests limited therapeutic value for phlebotomy.",
author = "Naseema Gangat and Oliveira, {Jennifer L.} and Hoyer, {James D.} and Patnaik, {Mrinal M.} and Animesh Pardanani and Ayalew Tefferi",
note = "Funding Information: We thank Virgil Fairbanks M.D for establishing the high oxygen affinity hemoglobin variant clinical and laboratory database at the Mayo Clinic, Rochester, MN. Publisher Copyright: {\textcopyright} 2021 Wiley Periodicals LLC.",
year = "2021",
month = dec,
day = "1",
doi = "10.1002/ajh.26375",
language = "English (US)",
volume = "96",
pages = "1647--1654",
journal = "American Journal of Hematology",
issn = "0361-8609",
publisher = "Wiley-Liss Inc.",
number = "12",
}
TY - JOUR
T1 - High-oxygen-affinity hemoglobinopathy-associated erythrocytosis
T2 - Clinical outcomes and impact of therapy in 41 cases
AU - Gangat, Naseema
AU - Oliveira, Jennifer L.
AU - Hoyer, James D.
AU - Patnaik, Mrinal M.
AU - Pardanani, Animesh
AU - Tefferi, Ayalew
N1 - Funding Information:We thank Virgil Fairbanks M.D for establishing the high oxygen affinity hemoglobin variant clinical and laboratory database at the Mayo Clinic, Rochester, MN.Publisher Copyright:© 2021 Wiley Periodicals LLC.
PY - 2021/12/1
Y1 - 2021/12/1
N2 - We describe presenting features, treatment strategies, and follow-up events involving 41 patients (median age 39 years, range 1–81; 54% males) with high oxygen affinity (HOA) hemoglobinopathy-associated erythrocytosis, seen at our institution (1973–2020). Thirty-four (83%) patients carried β-chain (13 Malmo, 4 Olympia, 3 San Diego, 2 Wood) and 7 (17%) α-chain (4 Dallas and one each Columbia-Missouri, Jackson, and Wayne) variants. Median (range) hemoglobin (Hgb)/hematocrit (Hct), serum erythropoietin and p50 were 18 g/dL/52.9% (16–21.9/48–66), 10.4 mIU (4–36.3), and 20 mmHg (12–25), respectively. Family history was documented in 24 patients and history of thrombosis in two (5%). Treatment included phlebotomy in 23 and antiplatelet therapy in 21 patients. At a median follow-up of 10 years, 23 (56%) patients reported one or more symptoms that were thought to be related to their increased Hct while thrombosis was documented in 10 (24%) patients. Neither Hgb/Hct level nor active phlebotomy showed a significant correlation with either thrombotic or nonthrombotic symptoms (p >.1 in all instances). Among 23 pregnancies recorded, 78% resulted in live births and no fetal loss was attributed to erythrocytosis. The current study does not implicate Hgb/Hct level as a major contributor of morbidity in HOA hemoglobinopathy-associated erythrocytosis and suggests limited therapeutic value for phlebotomy.
AB - We describe presenting features, treatment strategies, and follow-up events involving 41 patients (median age 39 years, range 1–81; 54% males) with high oxygen affinity (HOA) hemoglobinopathy-associated erythrocytosis, seen at our institution (1973–2020). Thirty-four (83%) patients carried β-chain (13 Malmo, 4 Olympia, 3 San Diego, 2 Wood) and 7 (17%) α-chain (4 Dallas and one each Columbia-Missouri, Jackson, and Wayne) variants. Median (range) hemoglobin (Hgb)/hematocrit (Hct), serum erythropoietin and p50 were 18 g/dL/52.9% (16–21.9/48–66), 10.4 mIU (4–36.3), and 20 mmHg (12–25), respectively. Family history was documented in 24 patients and history of thrombosis in two (5%). Treatment included phlebotomy in 23 and antiplatelet therapy in 21 patients. At a median follow-up of 10 years, 23 (56%) patients reported one or more symptoms that were thought to be related to their increased Hct while thrombosis was documented in 10 (24%) patients. Neither Hgb/Hct level nor active phlebotomy showed a significant correlation with either thrombotic or nonthrombotic symptoms (p >.1 in all instances). Among 23 pregnancies recorded, 78% resulted in live births and no fetal loss was attributed to erythrocytosis. The current study does not implicate Hgb/Hct level as a major contributor of morbidity in HOA hemoglobinopathy-associated erythrocytosis and suggests limited therapeutic value for phlebotomy.
UR - http://www.scopus.com/inward/record.url?scp=85117169151&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85117169151&partnerID=8YFLogxK
U2 - 10.1002/ajh.26375
DO - 10.1002/ajh.26375
M3 - Article
C2 - 34633117
AN - SCOPUS:85117169151
SN - 0361-8609
VL - 96
SP - 1647
EP - 1654
JO - American Journal of Hematology
JF - American Journal of Hematology
IS - 12
ER -
FAQs
What does a high oxygen affinity hemoglobin mean? ›
High-oxygen-affinity hemoglobins release oxygen at a lower rate than normal and thus create relative tissue hypoxia, which might result in compensatory erythrocytosis in approximately one third of affected patients.
What are the symptoms of high affinity hemoglobin? ›High oxygen affinity variant hemoglobins
Patients generally present with polycythemia, often accompanied by a ruddy complexion and mucosal erythema. The clinical course may be complicated by thrombotic events and/or symptoms of hyperviscosity, such as headaches, vertigo, tinnitus, and paresthesia in the extremities.
Variants that increase the affinity of hemoglobin for oxygen cause isolated erythrocytosis. Variants that decrease the affinity of hemoglobin for oxygen cause cyanosis and/or anemia. Usually, individuals with disorders of hemoglobin oxygen affinity do not require treatment.
What is high oxygen affinity hemoglobin polycythemia? ›High affinity Hb variants release oxygen in the tissue relatively slowly and create relative tissue hypoxia. This leads to increased production of Epo from kidneys which results in increased red blood cell mass and polycythemia.
Is erythrocytosis a blood disorder? ›Erythrocytosis, sometimes called polycythaemia, means having a high concentration of red blood cells in your blood. This makes the blood thicker and less able to travel through blood vessels and organs. Many of the symptoms of erythrocytosis are caused by this sluggish flow of blood.
What is the significance of this hemoglobin affinity difference? ›The higher the affinity of a given protein for oxygen, the harder it will be for that protein to release oxygen when the time comes. Thus, hemoglobin's lower affinity for oxygen serves it well because it allows hemoglobin to release oxygen more easily in the cells.
Should I worry if my hemoglobin is high? ›If you have a high hemoglobin count without other abnormalities, it's unlikely to indicate a related serious condition. Conditions that can cause a high hemoglobin count include: Congenital heart disease in adults.
What is the treatment of high hemoglobin? ›Medications: A doctor may prescribe medications to treat your elevated levels of hemoglobin. The course of treatment largely depends upon underlying health issues that caused a change in your hemoglobin count. Phlebotomy: In this treatment option, the doctor will use needles or syringes to remove blood from your body.
Can dehydration cause high hemoglobin and hematocrit? ›If a patient is severely dehydrated, the hemoglobin and hematocrit will appear higher than if the patient were normovolemic; if the patient is fluid overloaded, they will be lower than their actual level.
Which of the following causes an increase in hemoglobin o2 affinity? ›Changes in carbon dioxide (CO2) are associated with shifts in hemoglobin's oxygen affinity. Increases in CO2 decrease hemoglobin saturation, while decreases in CO2 increase hemoglobin saturation.
What 4 factors affect hemoglobin's affinity for oxygen? ›
The affinity of hemoglobin is affected by temperature, hydrogen ions, carbon dioxide, and intraerythrocytic 2,3-DPG, with all these factors mutually influencing each other.
What 5 factors affect the affinity of hemoglobin and oxygen? ›There are several important factors that affect the affinity of hemoglobin to oxygen as therefore affect the oxygen-hemoglobin dissociation curve. These factors include the (1) pH (2) temperature (3) carbon dioxide (4) 2,3-BPG and (5) carbon monoxide.
Does high hemoglobin always mean polycythemia? ›A high hemoglobin level is often an indicator of disease, including polycythemia vera, cancer, heart disease, lung disease, and kidney or liver disease. Smoking and high altitudes can also cause hemoglobin levels to increase. Hemoglobin levels can be checked with a routine blood test.
Is polycythaemia bad? ›If well controlled, polycythaemia shouldn't affect your life expectancy, and you should be able to live a normal life. People with PV can have a slightly lower life expectancy than normal due to the increased risk of problems, such as heart attacks and strokes.
Does high hemoglobin mean polycythemia vera? ›Polycythemia vera (PV) is a myeloproliferative neoplasm, and its diagnosis requires elevated hemoglobin level (>16.5 mg/dL in men and >16 mg/dL in women), bone marrow characteristics of PV (hypercellularity for age with trilineage growth), and presence of JAK2 (Janus kinase 2) mutations or subnormal erythropoietin ...
Can erythrocytosis be cured? ›Most causes of erythrocytosis can't be cured. Instead, treatment can help ease symptoms. With more serious causes of erythrocytosis, your provider may provide treatments to prevent potential complications, like blood clots.
What is the most common cause of erythrocytosis? ›Primary erythrocytosis — or autonomous production of excess erythrocytes — most commonly occurs due to polycythemia vera (PV), a myeloproliferative neoplastic process that may be asymptomatic or may present with thrombosis, constitutional or vasomotor symptoms, or splenomegaly.
What are the signs and symptoms of erythrocytosis? ›- Blurred vision.
- Headaches.
- Confusion.
- High blood pressure.
- Nosebleeds.
- Itching.
- Weakness.
- Tiredness.
- Carbon monoxide exposure.
- Dehydration.
- Drugs such as anabolic steroids or erythropoietin-stimulating agents.
- Living at a high altitude.
- Smoking.
Carbon monoxide has 210 times greater affinity for haemoglobin than oxygen1. A small environmental concentration will thus cause toxic levels of carboxyhaemoglobin.
Is the affinity of your hemoglobin for o2 higher or lower when you are exercising? ›
Blood entering capillaries of exercising muscles is acutely exposed to these changes, which causes a rapid decrease in Hb-O2 affinity.
What happens to hemoglobin during carbon monoxide poisoning? ›Carbon monoxide poisoning occurs as carbon monoxide mixes and binds with hemoglobin in the blood to form carboxyhemoglobin (COHb). When carbon monoxide binds to hemoglobin, less oxygen gets transported to body tissues and vital organs such as the brain and heart.
What does carbon monoxide poisoning of hemoglobin lead to? ›Carbon monoxide gas diffuses rapidly across the pulmonary capillary membrane, binding to hemoglobin with an affinity 200 times that of oxygen to form carboxyhemoglobin. By displacing oxygen, CO decreases the oxygen-carrying capacity and oxygen delivery to tissues, causing marked cellular hypoxia and acidosis.
Does carbon monoxide poisoning affect hemoglobin? ›Exposure to carbon monoxide impedes the blood's ability to carry oxygen to body tissues and vital organs. When carbon monoxide is inhaled, it combines with hemoglobin (an iron-protein component of red blood cells), producing carboxyhemoglobin (COHb), which greatly diminishes hemoglobin's oxygen-carrying capacity.
Does exercise lower red blood cells? ›Exercise can increase the total Hb and red blood cell mass, which increases oxygen carrying capacity so that with structured exercise the hemoglobin level in the blood which functions to bind oxygen in the blood and relax it throughout the body will also increase.
What level of hemoglobin is dangerously high? ›The threshold for a high hemoglobin count differs slightly from one medical practice to another. It's generally defined as more than 16.6 grams (g) of hemoglobin per deciliter (dL) of blood for men and 15 g/dL for women.
Can exercise lower hemoglobin? ›Hemoglobin level is decreased because aerobic exercise expands the baseline plasma volume; this reduces the concentration of red cells, which contain the hemoglobin. In other words, the naturally lower hemoglobin level of an endurance athlete is a dilutional pseudoanemia.