scholarly journals Family case hemolytic anemia due to abnormal unstable hemoglobin with low oxygen affinity (Hb Cheverly)

2019 ◽  
Vol 18 (3) ◽  
pp. 62-69
Author(s):  
E. L. Krivosheina ◽  
N. Yu. Koval ◽  
E. N. Egorova ◽  
M. A. Gorshkova ◽  
N. A. Karamyan ◽  
...  
Keyword(s):  
Oxygen Saturation ◽  
Pulse Oximetry ◽  
Hemolytic Anemia ◽  
Intensive Therapy ◽  
Clinical Manifestations ◽  
Oxygen Affinity ◽  
Personal Data ◽  
Low Oxygen ◽  
Unstable Hemoglobin ◽  
Low Oxygen Affinity

Hereditary hemolytic anemia caused by unstable hemoglobin is a rare pathology, characterized by variability of clinical manifestations. This disease is characterized by the hemolytic crises, which are frequently associated with infections or taking drugs that cause hemolysis. Age of debut depends on which of the globin chains mutation occurs. Among these diseases, hemolytic anemia associated with the presence of a structurally abnormal unstable hemoglobin with low oxygen affinity in the erythrocytes is a special group. With this type of abnormal hemoglobin, pulse oximetry demonstrates falsely low oxygen saturation of the blood, with increased oxygen delivery to the tissues. It is important to identify unstable hemoglobin in order to avoid the misinterpretation of low oxygen saturation during the pulse oximetry in emergency cases, surgeries or intensive therapy. The article presents an analysis of the family with hereditary hemolytic anemia due to unstable hemoglobin Cheverly. The main clinical and diagnostic markers of the disease are described in detail. Parents gave their permission for using personal data for clinical research and publications.

scholarly journals Hemoglobin Sunshine Seth: A Case Report of Low-Oxygen-Affinity Hemoglobinopathy

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Leah S. Heidenreich ◽  
Jennifer L. Oliveira ◽  
Peter J. Holmberg ◽  
Vilmarie Rodriguez
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Case Report ◽  
Oxygen Saturation ◽  
Pulse Oximetry ◽  
Congenital Heart ◽  
Oxygen Affinity ◽  
Low Oxygen ◽  
Peripheral Oxygen Saturation ◽  
Low Oxygen Affinity

Pulse oximetry is routinely used in the newborn nursery for clinical monitoring and to detect critical congenital heart disease. The differential diagnoses for reduced peripheral oxygen saturation in an infant include congenital heart disease, respiratory distress syndrome, transient tachypnea of the newborn, persistent pulmonary hypertension of the newborn, meconium aspiration syndrome, pneumonia, pneumothorax, and sepsis. The diagnostic evaluation for neonatal hypoxemia can be invasive and expensive. When this evaluation is unrevealing, other interventions may be tried without clear benefit to the patient, including, but not limited to, supplemental oxygen. Therefore, it is important to consider alternative, albeit rare, diagnoses, including hemoglobinopathies with abnormal oxygen binding properties. Mutations in the structure of alpha- and beta-globin chains can alter the affinity of hemoglobin for oxygen, and changes in oxygen affinity may result in changes in the oxygen saturation detected by pulse oximetry. These changes may or may not be of clinical significance. This case report describes Hemoglobin Sunshine Seth, a rare low-oxygen-affinity hemoglobin variant presenting as reduced peripheral oxygen saturation in an otherwise well-appearing infant male.

A NEW UNSTABLE HEMOGLOBIN VARIANT WITH LOW OXYGEN AFFINITY: Hb ILMENAU [β41(C7)Phe→Cys]

Hemoglobin ◽  
2002 ◽  
Vol 26 (2) ◽  
pp. 169-174 ◽  
Author(s):  
Claude Préhu ◽  
Lütje J. Behnken ◽  
Rüdiger Neumann ◽  
Jean Riou ◽  
Jean Kister ◽  
...  
Keyword(s):  
Oxygen Affinity ◽  
Low Oxygen ◽  
Hemoglobin Variant ◽  
Unstable Hemoglobin ◽  
Low Oxygen Affinity

HB Washtenaw [βT11(A8)VAL-PHE]: An Electrophoretically Silent, Unstable, Low Oxygen Affinity Variant Associated with Anemia and Chronic Cyanosis

Hemoglobin ◽  
1994 ◽  
Vol 18 (4-5) ◽  
pp. 285-295 ◽  
Author(s):  
K. Krishnan ◽  
F. Martinez ◽  
R. T. Wille ◽  
R. T. Jones ◽  
D. T. Shin ◽  
...  
Keyword(s):  
Oxygen Affinity ◽  
Low Oxygen ◽  
Low Oxygen Affinity

Purification, Crystallization and Preliminary X-Ray Diffraction Studies on Goat (Capra hircus) Hemoglobin - A Low Oxygen Affinity Species

2009 ◽  
Vol 16 (4) ◽  
pp. 454-456 ◽  
Author(s):  
Ponnuraj Moorthy ◽  
Kamariah Neelagandan ◽  
Moovarkumudalvan Balasubramanian ◽  
Mondikalipudur Ponnuswamy
Keyword(s):  
Oxygen Affinity ◽  
Capra Hircus ◽  
Low Oxygen ◽  
X Ray Diffraction ◽  
X Ray ◽  
Hemoglobin A ◽  
Low Oxygen Affinity

scholarly journals Sequestro Leucocitário de Oxigénio

2016 ◽  
Vol 29 (5) ◽  
pp. 343
Author(s):  
Miguel Pinto da Costa ◽  
Henrique Pimenta Coelho
Keyword(s):  
Oxygen Saturation ◽  
Pulse Oximetry ◽  
Arterial Oxygen Saturation ◽  
Gas Analysis ◽  
Lymphocytic Leukemia ◽  
Red Blood Cell Transfusion ◽  
Clinical State ◽  
Arterial Oxygen ◽  
Low Oxygen ◽  
Arterial Blood

<p>The authors present a case of a 60-year-old male patient, previously diagnosed with B-cell chronic lymphocytic leukemia, who was admitted to the Emergency Room with dyspnea. The initial evaluation revealed severe anemia (Hgb = 5.0 g/dL) with hyperleukocytosis (800.000/µL), nearly all of the cells being mature lymphocytes, a normal chest X-ray and a low arterial oxygen saturation (89%; pulse oximetry). After red blood cell transfusion, Hgb values rose (9.0 g/dL) and there was a complete reversion of the dyspnea. Yet, subsequent arterial blood gas analysis, without the administration of supplemental oxygen, systematically revealed very low oxygen saturation values (~ 46%), which was inconsistent with the patient’s clinical state and his pulse oximetry values (~ 87%), and these values were not corrected by the administration of oxygen via non-rebreather mask. The investigation performed allowed to establish the diagnosis of oxygen leukocyte larceny, a phenomenon which conceals the true oxygen saturation due to peripheral consumption by leukocytes.</p>

scholarly journals Hemoglobin Titusville: a rare low oxygen affinity hemoglobinopathy

2017 ◽  
Vol 5 (6) ◽  
pp. 1011-1012 ◽  
Author(s):  
Senan John Yasar ◽  
Vivian Irene Ravn Berg ◽  
Asim Ahmad ◽  
Donald Doll
Keyword(s):  
Oxygen Affinity ◽  
Low Oxygen ◽  
Low Oxygen Affinity
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