Presentation
The present case is significant because of the paucity of previously described focal intra-abdominal infections caused by L. monocytogenes, particularly as a postsplenectomy pathogen. [ncbi.nlm.nih.gov]
[…] and why presents during second 6 mos of life as levels of HbF taper off Presentation of beta-thalassemia major hepatosplenomegaly, jaundice, expansion of erythroid marrow, increased susceptibility to infection Rx for beta-thalassemia major regular, lifelong [memorize.com]
Absent (may be present with severe extravascular hemolysis) Hemoglobinuria Present Absent Urine hemosiderin Present Absent Hemopexin-heme Present Absent (may be present with severe extravascular hemolysis) Methemalbumin Present Absent Morphology Schistocytes [clinicalgate.com]
The case is described of a 9-month-old infant with congenital HIV-1 infection who presented with severe autoimmune hemolysis and a high cytomegalovirus viral plasma load. [link.springer.com]
Symptoms may be present when swallowing cold foods or drinks. 35. [slideshare.net]
Entire Body System
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Fatigue
[…] virus, cytomegalovirus, HIV, and hepatitis Symptoms of AIHA People with acquired autoimmune hemolytic anemia have symptoms that include: Chills Fast heartbeat, known as tachycardia Pale skin that may start to yellow Shortness of breath Weakness and fatigue [webmd.com]
All hemolytic anemias feature varying degrees of fatigue, pallor, and weakness (from asymptomatic disease to life-threatening hemolytic crisis ), although some diseases have more specific findings (e.g., venous thrombosis in paroxysmal nocturnal hemoglobinuria [amboss.com]
The disease is characterized by symptoms due to the anemia including fatigue, exertional dyspnea, and, more rarely, jaundice and dark urine in case of severe hemolysis. If the disease is severe, fever, chest pain, syncope or heart failure may occur. [orpha.net]
Signs and symptoms may include fatigue, dizziness, heart palpitations, pale skin, headache, confusion, jaundice, and a spleen or liver that is larger than normal. [nhlbi.nih.gov]
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HELLP Syndrome
[…] plant poisons such as aesculin) Trauma Mechanical (from heart valves, extensive vascular surgery, microvascular disease, repeated mechanical vascular trauma) Microangiopathic hemolytic anemia (a specific subtype with causes such as TTP, HUS, DIC and HELLP [en.wikipedia.org]
SYNDROME HEMOLYSIS ELEVATED LIVER ENZYMES LOW PLATLET COUNTS 62 HELLP SYNDROME 1 IN 1OOO PREGNANCIES SEEN IN PRESENCE OF ECLAMPSIA, PREECLAMPSIA, AND PLACENTAL ABRUPTION MAY EXTEND UP TO 6 DAYS POSTPARTUM 63 HELLP SYNDROME RUQ AND EPIGASTRIC PAIN – SEEN [slideplayer.com]
[…] plant poisons such as aesculin ) Trauma Mechanical (from heart valves, extensive vascular surgery, microvascular disease, repeated mechanical vascular trauma) Microangiopathic hemolytic anemia (a specific subtype with causes such as TTP, HUS, DIC and HELLP [ipfs.io]
syndrome (named for its characteristics, which include hemolysis, elevated liver enzymes, and low platelet count) In some instances, hemolytic anemia is the result of taking certain medications. [healthline.com]
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Virilization
[…] long-lasting remissions in some patients; 98, 99 possible mechanisms include reduction in RBC-bound C3d, immunomodulation by alteration of T-cell subsets and downregulation of FcR in the reticuloendothelial (RE) system. 100 Side-effects of danazol include virilization [clinicalgate.com]
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Increased Susceptibility to Infections
susceptibility to infection Rx for beta-thalassemia major regular, lifelong transfusions and rx for iron overload Smear in beta-thalassemia major low MCV, microcytic, hypochromic cells with increased RDW Clinical effect of sickle cell- beta-thalassemia [memorize.com]
Gastrointestinal
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Abdominal Fullness
When destruction persists for a few months or longer, the spleen may enlarge, resulting in a sense of abdominal fullness and, occasionally, discomfort. In people with cold antibody hemolytic anemia, the hands and feet may be cold or bluish. [merckmanuals.com]
Skin
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Blister
Hemoglobinuria Present Absent Urine hemosiderin Present Absent Hemopexin-heme Present Absent (may be present with severe extravascular hemolysis) Methemalbumin Present Absent Morphology Schistocytes, spherocytes, agglutination Spherocytes, bite cells, blister [clinicalgate.com]
Blood film spherocytes (AIHA, hereditary spherocytosis) helmet cells blister cells (oxidative damage in G6PD deficiency) schistocytes (TTP or DIC with thrombocytopenia) or heart valve hemolysis (with thrombocytopenia) acanthocytes (liver disease) Heinz [lifeinthefastlane.com]
Musculoskeletal
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Swelling of Hand
[…] of hands and feet) Heart Enlargement Growth Retardation Delayed Sexual Development 29 Dactylitis 30 Sickle Cell Crisis Severe pain caused by blocked blood flow Triggered by Infection, Dehydration, Fatigue, or Emotional Stress Can last up to 5 days 31 [slideplayer.com]
Neurologic
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Headache
Attacks are precipated on exposure to cold and assoc with hemoglobinemia, hemoglobinuria, chills and fever, back, leg and abdominal pain, headache and malaise. 42. TREATMENT. Maintain patient in warm environment. Splenectomy not of value. [slideshare.net]
Signs and symptoms may include fatigue, dizziness, heart palpitations, pale skin, headache, confusion, jaundice, and a spleen or liver that is larger than normal. [nhlbi.nih.gov]
Infection, Dehydration, Fatigue, or Emotional Stress Can last up to 5 days 31 Splenic Sequestration Spleen tried to remove abnormal cells Becomes enlarged and causes pain Autosplenectomy occurs Usually not seen in adults 32 Symptoms of Anemia Tiredness Headaches [slideplayer.com]
People with paroxysmal cold hemoglobinuria may have severe pain in the back and legs, headache, vomiting, and diarrhea. The urine may be dark brown. Once blood tests show a person has anemia, doctors look for the cause. [merckmanuals.com]
Workup
An array of laboratory tests are available for detecting hemolysis, and specialized tests may be indicated to diagnose the cause for hemolysis (see Workup ). [emedicine.medscape.com]
Microbiology
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Listeria Monocytogenes
We report a case of an intra-abdominal abscess caused by Listeria monocytogenes in a postoperative patient with Evans syndrome (acquired hemolytic anemia and thrombocytopenia). [ncbi.nlm.nih.gov]
Biopsy
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Bone Marrow with Erythroid Hyperplasia
Bone marrow smear erythroid hyperplasia 78 Diagnosis of hemolytic syndrome: 1. Anemia 2. [slideplayer.com]
Treatment
Reticulocytosis 3 Diagnosis of hemolytic syndrome: Anemia Reticulocytosis Indirect hyperbilirubinemia 79 Autoimmune hemolytic anemia - diagnosis positive Coombs’ test Treatment: steroids splenectomy immunosupressive agents transfusion [slideplayer.com]
TREATMENT If mild hemolysis no treatment is required. Remove the underlying cause. If significant hemolysis Treat with Prednisolone(1mg/kg/day) Hb rises within 3 or 4 days and in most by 1 to 2 weeks. [slideshare.net]
If your AIHA is mild, you may not need any treatment at all. Doctors usually first prescribe steroids, such as hydrocortisone or prednisone, to stop your immune system from attacking your red blood cells. [webmd.com]
If no response to corticosteroids is observed by the end of the first 3 weeks, continued therapy as sole treatment is usually ineffective. [clinicalgate.com]
Prognosis
Prognosis depends on the underlying condition. The documents contained in this web site are presented for information purposes only. [orpha.net]
Prognosis AIHA is transient in children following viral infection. Usually chronic in adults with exacerbations and remissions 27. Warm AIHA 28. COLD ANTIBODY IMMUNOHEMOLYTIC ANEMIA. [slideshare.net]
Synonyms and Keywords: Haemolytic anaemia Overview Historical Perspective Classification Pathophysiology Causes Epidemiology and Demographics Risk Factors Screening Differentiating Hemolytic anemia from other Diseases Natural History, Complications and Prognosis [wikidoc.org]
Hemoglobin Increased retic count White cell count increased WBC shift to the left 36 37 Hgb Electrophoresis Amino acids in globin chains have different charges Separates hemoglobin according to charge 90% Hgb S, 10% Hgb F, small fraction of Hgb A2 38 39 Prognosis [slideplayer.com]
Treatments and Prognosis for Hemolytic Anemia Doctors who treat hemolytic anemia will first focus on returning the patient's red blood cell count to as close to normal as possible and halting or slowing the destruction of red blood cells. [everydayhealth.com]
Etiology
Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]
In many cases, a fundamental pathologic process such as lymphosarcoma or leukemia can be found (symptomatic 1 2 3 or secondary types), but most of the cases have no well defined etiology — that is, they are "idiopathic." [nejm.org]
The occurrence of definite and sustained leukopenia with neutropenia and thrombocytopenia in several patients with hemolytic disease due to an immune body agent raises questions as to the etiology of classic thrombocytopenic purpura and of splenic neutropenia [bloodjournal.org]
Enzyme defects Glucose-6-phosphate dehydrogenase deficiency Pyruvate kinase deficiency Pathophysiology : autosomal recessive defect of pyruvate kinase Etiology : : Glucose is the only energy source for RBCs. [amboss.com]
Etiology and Pathogenesis Overview Taken together, AIHA represents the most common cause of acquired hemolytic anemia; however, a number of distinct insults can trigger generation of these anti-erythrocyte antibodies. [pathwaymedicine.org]
Epidemiology
It discusses the biological basis of disease at genetic, molecular, cellular, and epidemiologic levels. [books.google.com]
Summary Epidemiology Warm auto-antibodies are responsible for 60/70% of AIHA, whose annual incidence is estimated to be between 1/35,000-1/80,000 in North America and Western Europe. [orpha.net]
M.D. [1] Shyam Patel [2] Synonyms and Keywords: Haemolytic anaemia Overview Historical Perspective Classification Pathophysiology Causes Epidemiology and Demographics Risk Factors Screening Differentiating Hemolytic anemia from other Diseases Natural [wikidoc.org]
Epidemiology Risk factors are variable and depend on the underlying cause. [patient.info]
Epidemiology Hemolytic anemia represents approximately 5% of all anemias. Acute AIHA is relatively rare, with an incidence of one to three cases per 100,000 population per year. [27] Hemolytic anemias are not specific to any race. [emedicine.medscape.com]
Pathophysiology
[…] of hemolytic severity LDH memorize Hereditary Spherocytosis Question Answer What ethnicity gets the most hereditary spherocytosis northern European Inheritance of hereditary spherocytosis autosomal dominant (rare, very severe recessive form as well) Pathophysiology [memorize.com]
Enzyme defects Glucose-6-phosphate dehydrogenase deficiency Pyruvate kinase deficiency Pathophysiology : autosomal recessive defect of pyruvate kinase Etiology : : Glucose is the only energy source for RBCs. [amboss.com]
Paroxysmal Nocturnal Hemoglobinuria (PNH) Discuss the molecular and pathophysiologic defects in paroxysmal nocturnal hemoglobinuria (PNH) and explain the test used to diagnose this disorder. List complications of PNH. [hematology.org]
M.D. [1] Shyam Patel [2] Synonyms and Keywords: Haemolytic anaemia Overview Historical Perspective Classification Pathophysiology Causes Epidemiology and Demographics Risk Factors Screening Differentiating Hemolytic anemia from other Diseases Natural [wikidoc.org]
Pathophysiology - red cell membrane protein defects (spectrin deficiency) resulting cytoskeleton instability 2. Familly history 3. Clinical features - splenomegaly 4. [slideplayer.com]
Prevention
Author affiliations Departments of Preventive Medicine and Medicine, Washington University School of Medicine, St. Louis, Mo. [karger.com]
Laboratory features - hemolytic anemia - blood smear-microspherocytes abnormal osmotic fragility test positive autohemolysis test prevention of increased autohemolysis by including glucose in incubation medium 5. [slideplayer.com]
Prevention It is not possible to prevent some types of AIHA, but doctors can monitor people who have a viral infection or who use certain medications, to ensure that AIHA does not develop. [medicalnewstoday.com]
Causes include RBC membrane abnormalities such as bound immunoglobulin, or physical abnormalities restricting RBC deformability that prevent egress from the spleen. Extravascular hemolysis is characterized by spherocytes. [med-ed.virginia.edu]
Recognizing these symptoms is important in order to prevent fatal outcomes. [consumerhealthdigest.com]