These multimers trap platelets, causing microthrombi and RBC destruction by shearing, creating schistocytes. TTP is life-threatening and requires timely diagnosis and treatment.
Additional laboratory findings include a negative DAT result and normal coagulation testing. Although plasma exchange is superior, fresh frozen plasma infusion is beneficial and should be started if transfer to a plasma exchange—capable center is delayed. Information from reference Hemolytic uremic syndrome HUS is characterized by MAHA and acute kidney injury, and commonly thrombocytopenia and neurologic dysfunction.
There is an atypical HUS that also does not have the prodrome, is caused by complement dysregulation and not infection, and can be hereditary.
Exacerbations can be triggered by an upper respiratory tract infection. Inadequately cooked ground beef is the primary source of STEC infection, but fruits, vegetables, poultry, and contaminated drinking water have also been implicated. In STEC-HUS, the Shiga toxin is absorbed and attaches to specific receptors, most expressed in the glomerulus and brain in children, causing endothelial cell damage, which initiates a cascade resulting in large von Willebrand factor multimers that induce MAHA.
Disseminated intravascular coagulation causes prolonged coagulation studies, positive d -dimer test results, and decreased fibrinogen levels. Drug-induced TMA occurs when a compound causes the formation of platelet microthrombi, resulting in MAHA through induced antibodies or direct toxicity.
These antibodies interact strongly only in the presence of the drug. The clinical features are similar to those of other MAHA syndromes. Quinine, cyclosporine Sandimmune , and tacrolimus Prograf constituted more than one-half of all drug-induced TMA cases 20 Table 4. The management of drug-induced TMA includes discontinuing the offending agent and providing supportive care; plasma exchange is not beneficial, except in the case of ticlopidine.
This results in methemoglobinemia i. G6PD is integral to these protective systems, and when it is deficient, oxidative insults may cause hemolysis.
G6PD deficiency is an X-linked disorder and is common in individuals of Mediterranean and African descent. The diagnosis is made by G6PD activity testing, although this may be normal during or just after a hemolytic episode. Treatment is discontinuation of the drug and supportive care.
Methylene blue is indicated for the treatment of severe methemoglobinemia from a non-G6PD cause, but it is possibly harmful and contraindicated in persons with G6PD deficiency.
A rapid onset of anemia or significant hyperbilirubinemia i. Hemolytic disease of the fetus and newborn is an alloimmune hemolysis caused by maternal antibodies in the neonate's plasma, is most commonly anti-Rh, and is DAT-positive. Hemolytic disease of the fetus and newborn is treated with supportive care and hyperbilirubinemia management. Hereditary spherocytosis is the most common inherited membranopathy and is caused by one of several defective proteins.
In severe cases, it can cause hemolysis in the neonatal period but typically presents later as chronic hemolysis. The mutations are largely autosomal dominant, making the family history important. In the setting of a DAT-negative hemolysis and spherocytes on peripheral blood smear, an increased mean corpuscular hemoglobin concentration—to—mean corpuscular volume ratio greater than 0.
Inherited hemoglobinopathies, such as sickle cell disease and thalassemias, can present in the neonatal period or later, depending on their severity. This article updates a previous article on this topic by Dhaliwal, et al.
Relevant UpToDate articles were also accessed to provide references. An Essential Evidence Plus search was provided to the authors as well. The Cochrane database was also queried. Search dates: April and May , and March Figures 2 and 3 provided by Lyndon P. The views expressed in this article are those of the authors and do not necessarily reflect the official policy of the Department of the Army, the Department of Defense, or the U.
Already a member or subscriber? Log in. Interested in AAFP membership? Learn more. ADAM C. Reprints are not available from the authors. Extrinsic nonimmune hemolytic anemias.
Hematology: Basic Principles and Practice. Philadelphia, Pa. Bain BJ. Diagnosis from the blood smear. N Engl J Med. Autoimmune hemolytic anemia. Transfusion of blood and blood products: indications and complications. Garratty G. Drug-induced immune hemolytic anemia. One center's experience: the serology and drugs associated with drug-induced immune hemolytic anemia—a new paradigm.
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Blue cures blue but be cautious. J Pharm Bioallied Sci. J Perinatol. Hyperbilirubinemia among African American, glucosephosphate dehydrogenase-deficient neonates. American Academy of Pediatrics Subcommittee on Hyperbilirubinemia. Management of hyperbilirubinemia in the newborn infant 35 or more weeks of gestation [published correction appears in Pediatrics. A pediatrician's practical guide to diagnosing and treating hereditary spherocytosis in neonates.
Sickle cell anemia is an inherited disorder in which abnormal hemoglobin the red pigment inside red blood cells is produced. The abnormal hemoglobin causes red blood cells to assume a sickle shape, like the ones seen in this photomicrograph. These crescent or sickle-shaped red blood cells RBCs are present with Sickle cell anemia, and stand out clearly against the normal round RBCs.
These abnormally shaped cells may become entangled and block blood flow in the small blood vessels capillaries. The bone marrow is mostly responsible for making new red cells. Bone marrow is the soft tissue in the center of bones that helps form all blood cells. Hemolytic anemia occurs when the bone marrow isn't making enough red cells to replace the ones that are being destroyed.
Do you feel tired and listless? Do you find your mind drifting during the day? Do you get dizzy or short of breath whenever you climb the stairs? There are a few possible reasons for the way you feel, but you could have anemia. You could even have anemia without noticing any symptoms at all. Anemia is a problem with hemoglobin, a substance in red blood cells that carries oxygen throughout your body.
Without enough hemoglobin, your heart and other organs can't get the oxygen they need to work. When your organs slow down, you slow down and you start feeling tired and listless. Many different health conditions can cause anemia, from heavy blood loss during a woman's period, to pregnancy, to an underactive thyroid gland.
Healthy red blood cells are made in your bone marrow, the soft tissue in the middle of your bones. Any disease that damages blood marrow, such as lymphoma or leukemia, can also affect your red blood cell production. Anemia can also be caused by an immune system problem that damages red blood cells, or surgery to the stomach or intestines.
How do you know if you have anemia? You may feel tired, dizzy, and have trouble concentrating. You may get sick more often. People with anemia often complain of chest pain, headaches, or shortness of breath. Your skin might look pale, like you haven't seen the sun for months. Because these can also be symptoms of other conditions, your doctor will confirm that you have anemia by taking a blood test to check your red blood cell count and hemoglobin level. Acquired hemolytic anemia is not something you are born with.
You develop the condition later. What causes hemolytic anemia? Different diseases, conditions, or factors can cause each type: Inherited With the inherited type, parents pass the genes for the condition on to their children.
Acquired With this type of anemia, you are not born with a certain condition. This may happen because of: Certain infections, which may be viral or bacterial Medicines, such as penicillin, antimalarial medicines, sulfa medicines, or acetaminophen Blood cancers Autoimmune disorders, such as lupus, rheumatoid arthritis, or ulcerative colitis Certain tumors An overactive spleen hypersplenism Mechanical heart valves that may damage red blood cells as they leave the heart A severe reaction to a blood transfusion Some types of acquired hemolytic anemia are short-term temporary and go away over several months.
What are the symptoms of hemolytic anemia? How is hemolytic anemia diagnosed? Your provider may also order the following tests: Complete blood count CBC. This test measures many different parts of your blood. Other blood tests. If the CBC test shows that you have anemia, you may have other blood tests. These can find out what type of anemia you have and how serious it is.
Urine test. This can check for hemoglobin a protein in red blood cells and iron. Bone marrow aspiration or biopsy. This involves taking a small sample of bone marrow fluid aspiration or solid bone marrow tissue called a core biopsy. The sample is usually taken from the hip bones. It is checked for the number, size, and maturity of blood cells or abnormal cells. How is hemolytic anemia treated? Your healthcare provider will create a treatment plan based on: Your age, overall health, and medical history How sick you are The cause of the disease How well you handle certain medicines, treatments, or therapies If your condition is expected to get worse Your opinion or preference The treatment for hemolytic anemia will vary depending on the cause of the illness.
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