Haemolytic anaemia

Your doctor will diagnose hemolytic anemia based on your medical and family histories, a physical exam, and test results. Specialists Involved Primary care doctors, such as a family doctor or pediatrician, may help diagnose and treat hemolytic anemia. Your primary care doctor also may refer you to a hematologist. This is a doctor who specializes in diagnosing and treating blood diseases and disorders. Doctors and clinics that specialize in treating inherited blood disorders, such as If your hemolytic anemia is inherited, you may want to consult a genetic counselor. A counselor can help you understand your risk of having a child who has the condition. He or she also can explain the choices that are available to you. Medical and Family Histories To find the cause and severity of hemolytic anemia, your doctor may ask detailed questions about your symptoms, personal medical history, and your family medical history. He or she may ask whether: • You or anyone in your family has had problems with • You've recently had any illnesses or medical conditions • You take any medicines, and which ones • You've been exposed to certain chemicals or substances • You have an artificial heart valve or other medical device that could damage your red blood cells Physical Exam Your doctor will do a physical exam to check for signs of hemolytic anemia. He or she will try to find out how severe the condition is and what's causing it. The exam may include: • Checking for jaundice (a yellowish color...

Acknowledgment NORD gratefully acknowledges Elizabeth L. Kugelmann, MMSc, NORD Editorial Intern from the Emory University Genetic Counseling Training Program and Cecelia A. Bellcross, PhD, MS, CGC, Associate Professor, Director, Genetic Counseling Training Program, Emory University School of Medicine, for assistance in the preparation of this report. Disease Overview Summary Hereditary nonspherocytic hemolytic anemia refers to a group of conditions for which the main feature is the premature destruction of red blood cells. Red blood cells move oxygen throughout the body. Premature destruction of red blood cells is called hemolytic anemia. “Nonspherocytic” means the red blood cells are not sphere-shaped like normal red blood cells, and “hereditary” means the conditions are inherited. There are over 16 conditions that fall under the category of hereditary nonspherocytic hemolytic anemia, but they all share these common features. For some people, symptoms are present at birth, but for others, symptoms do not appear until adulthood. The most common forms of hereditary nonspherocytic hemolytic anemia are G6PD deficiency ( • • • • Next section > • Signs & Symptoms Symptoms People with hereditary nonspherocytic hemolytic anemia may experience yellowing of the skin (jaundice), tiredness, a large spleen (splenomegaly) and/or liver (hepatomegaly). Lab findings People with anemia may have the following results in bloodwork: increase in immature red blood cells (reticulocytosis), dec...

Autoimmune hemolytic anemia (AIHA) is a rare immune disorder. It happens when your body mistakes red blood cells as foreign substances and attacks them. Treatments include medication, surgery or, in rare cases, a blood transfusion. AIHA is highly manageable, but can be fatal if left untreated. Prompt care is critical. Overview What is autoimmune hemolytic anemia? Autoimmune hemolytic anemia (AIHA) occurs when your AIHA is highly manageable, but it can be fatal if left untreated. Immediate intervention is essential. What’s the difference between primary and secondary autoimmune hemolytic anemia? If your AIHA develops without the obvious presence of an underlying condition, it’s called primary AIHA. Secondary AIHA is when it’s linked to another condition, such as a viral illness, other autoimmune diseases, medication or underlying blood cancer (such as lymphoma). What are the types of autoimmune hemolytic anemia? There are two main types of autoimmune hemolytic anemia: warm autoimmune hemolytic anemia and cold autoimmune hemolytic anemia. This classification depends on the type of antibodies involved in the disease. Warm autoimmune hemolytic anemia The most common type of AIHA, warm autoimmune hemolytic anemia, involves IgG antibodies, which bind red blood cells at normal body temperature. Generally, symptoms occur gradually over the course of several weeks. In some cases, however, they can develop within days. Cold autoimmune hemolytic anemia Affecting 10% to 20% of cases, ...

Microangiopathic hemolytic anemia (MAHA) with thrombocytopenia, suggests a thrombotic microangiopathy (TMA), linked with thrombus formation affecting small or larger vessels. In cancer patients, it may be directly related to the underlying malignancy (initial presentation or progressive disease), to its treatment, or a separate incidental diagnosis. It is vital to differentiate incidental thrombotic thrombocytopenia purpura or atypical hemolytic uremic syndrome in cancer patients presenting with a TMA, as they have different treatment strategies, and prompt initiation of treatment impacts outcome. In the oncology patient, widespread microvascular metastases or extensive bone marrow involvement can cause MAHA and thrombocytopenia. A disseminated intravascular coagulation (DIC) picture may be precipitated by sepsis or driven by the cancer itself. Cancer therapies may cause a TMA, either dose-dependent toxicity, or an idiosyncratic immune-mediated reaction due to drug-dependent antibodies. Many causes of TMA seen in the oncology patient do not respond to plasma exchange and, where feasible, treatment of the underlying malignancy is important in controlling both cancer-TMA or DIC driven disease. Drug-induced TMA should be considered and any putative causal agent stopped. We will discuss the differential diagnosis and treatment of MAHA in patients with cancer using clinical cases to highlight management principles. Microangiopathic hemolytic anemia (MAHA) refers to a subgroup o...

Hemolytic anemia is defined by the premature destruction of red blood cells, and can be chronic or life-threatening. It should be part of the differential diagnosis for any normocytic or macrocytic anemia. Hemolysis may occur intravascularly, extravascularly in the reticuloendothelial system, or both. Mechanisms include poor deformability leading to trapping and phagocytosis, antibody-mediated destruction through phagocytosis or direct complement activation, fragmentation due to microthrombi or direct mechanical trauma, oxidation, or direct cellular destruction. Patients with hemolysis may present with acute anemia, jaundice, hematuria, dyspnea, fatigue, tachycardia, and possibly hypotension. Laboratory test results that confirm hemolysis include reticulocytosis, as well as increased lactate dehydrogenase, increased unconjugated bilirubin, and decreased haptoglobin levels. The direct antiglobulin test further differentiates immune causes from nonimmune causes. A peripheral blood smear should be performed when hemolysis is present to identify abnormal red blood cell morphologies. Hemolytic diseases are classified into hemoglobinopathies, membranopathies, enzymopathies, immune-mediated anemias, and extrinsic nonimmune causes. Extrinsic nonimmune causes include the thrombotic microangiopathies, direct trauma, infections, systemic diseases, and oxidative insults. Medications can cause hemolytic anemia through several mechanisms. A rapid onset of anemia or significant hyperbili...