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65 Possible Causes for Congenital Nonspherocytic Hemolytic Anemia, Erythrocytes Decreased, Splenomegaly

  • Hereditary Spherocytosis

    Due to the membrane defect, there is increased fragility, hemolytic anemia, marked splenomegaly and hyperbilirubinemia.[ncbi.nlm.nih.gov] Defects in these proteins lead to a loss in RBC membrane cohesion and membrane surface area, resulting in erythrocyte sphering, decreased deformability and premature destruction[orpha.net] Stability studies with blood from HS patients showed a significant decrease in hyperchromic erythrocytes after 6 h storage.[ncbi.nlm.nih.gov]

  • Autoimmune Hemolytic Anemia

    Examination demonstrated scleral icterus, splenomegaly, and anemia. By peripheral blood smear, the patient was diagnosed with Plasmodium ovale.[ncbi.nlm.nih.gov] Mechanisms of actions include suppression of autoantibody production, reduction in autoantibody affinity, and decreased destruction of erythrocytes by splenic macrophages,[doi.org] The diagnosis of HLH after SOT is especially difficult, as several of the diagnostic criteria, including fever, splenomegaly, and cytopenias, are nonspecific and can be seen[ncbi.nlm.nih.gov]

  • Pyruvate Kinase Deficiency

    She is doing well with mild splenomegaly.[ncbi.nlm.nih.gov] decreased erythrocyte pyruvate kinase (PK) activity.[ncbi.nlm.nih.gov] In this later group of "congenital nonspherocytic hemolytic anemias" splenectomy has often been performed but usually does not produce a clinical cure.[nejm.org]

  • Congenital Hemolytic Anemia

    Children with massive splenomegaly had similar outcomes compared with children without massive splenomegaly.[ncbi.nlm.nih.gov] Phosphatidylserine internalization in erythrocytes with the mutant ATP11C was decreased 10-fold compared to that of the control, functionally establishing that ATP11C is a[ncbi.nlm.nih.gov] This group is sometimes called congenital nonspherocytic (hemolytic) anemia, which is a term for a congenital hemolytic anemia without spherocytosis, and usually excluding[en.wikipedia.org]

  • Traumatic Hemolytic Anemia

    Splenomegaly and Hypersplenism (link accessible from “Related Topics” below) On a microscopic level the hematologist can see red blood cell fragments with odd shapes in a[nethealthbook.com] […] and the inability of bone marrow to compensate for the decreased life span.[diagnose-me.com] Physical findings include orthostatic hypotension, tachycardia, jaundice, icteris, and splenomegaly.[thebigl.co]

  • Congenital Dyserythropoietic Anemia

    CDA I patients have a moderate macrocytic anemia with frequent splenomegaly and occasional hepatomegaly.[orpha.net] We also found that erythrocyte anion exchanger 1 protein is decreased in CDA-II homozygotes and obligate carriers alike.[ncbi.nlm.nih.gov] , type I Congenital dyserythropoietic anemia, type II Congenital dyserythropoietic anemia, type III Congenital nonspherocytic hemolytic anemia due to inborn error of metabolism[icd9data.com]

  • Glycogen Storage Disease Type 1

    Ultrasound revealed a small, cirrhotic liver, portal vein thrombosis, and splenomegaly.[adc.bmj.com] Phk activity in erythrocytes was markedly decreased (12% residual activity) while the parents' erythrocyte Phk activities were in the heterozygote range (mother: 56%; father[academic.oup.com] Hepatomegaly, usually without enlargement of the spleen (splenomegaly), begins to develop in fetal life and is usually noticeable in the first few months of life.[en.wikipedia.org]

  • Hypersplenism

    RESULTS: While 38 patients displayed splenomegaly, 121 showed normal spleen volumes at 6 months after LDLT (LDLT).[ncbi.nlm.nih.gov] As a disorder, hypersplenism typically involves a distended spleen (splenomegaly), decreased levels of leukocytes, erythrocytes and platelets, shorter platelet and erythrocyte[symptoma.com] […] abscess (rare) Splenectomy sometimes indicated Elliptocytosis with hemolysis Nonspherocytic congenital hemolytic anemias Hodgkin’s disease (for staging) Idiopathic myelofibrosis[for-surgeons.com]

  • Toxic Hemolytic Anemia

    Splenomegaly. Venous thrombosis occasionally. 15. Severe form. There is severe hemolysis associated with hemoglobinemia,hemoglobinuria and shock.[slideshare.net] […] and the inability of bone marrow to compensate for the decreased life span.[diagnose-me.com] Hemolytic anemia in splenomegaly. Paroxysmal nocturnal hemoglobinuria. Anemia due to acquired RBCs membrane abnormality. Secondary to liver and kidneys diseases.[labpedia.net]

  • Acquired Toxic Hemolytic Anemia

    Pediatricians frequently fail to order a reticulocyte count or detect splenomegaly on physical examination before referring a patient with anemia.[pedsinreview.aappublications.org] Premature destruction of red cells occurs through two primary mechanisms: (1) decreased erythrocyte deformability that leads to red-cell sequestration and extravascular haemolysis[oxfordmedicine.com] Congenital Nonspherocytic Hemolytic Anemia includes any of a group of inherited anemias characterized by shortened red cell survival, lack of spherocytosis, and normal osmotic[diagnose-me.com]

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