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Hematology Exam 3

Hemoglobinopathies, Thalassemias, Parasitic Infections of RBCs, Lec 8

TermDefinition
What are the signs, symptoms, mutations, morphology, and Hemoglobin electrophoresis of SICKLE CELL DISEASE? (Hgb S) SYMPTOMS: sickled cells accumulate in kidney, lungs, brain, and spleen. MUTATIONS: only 1 copy of Hgb S gene where Valine is subbed for Glutamic Acid at position 6, or TWO copies of Hgb S ELECTROPHORESIS: 80% Hgb S and 20% F AFRICANS/AF. AMERICANS
What are the signs, symptoms, mutations, morphology, and Hemoglobin electrophoresis of Hgb C DISEASE? SYMPTOMS: Hemolytic anemia with splenomegaly and abdominal discomfort MUTATIONS: Lysine is subbed for Glutamic Acid at position 6 in beta chain ELECTROPHORESIS: wash/suspend cells in sodium citrate AFRICANS/AF. AMERICANS
What are the signs, symptoms, mutations, morphology, and Hemoglobin electrophoresis of Hgb SC DISEASE? SYMPTOMS: less anemia and more mild symptoms than SS disease MUTATIONS: NO TRAIT ELECTROPHORESIS: Hgb F is less than SS disease, crystals poke out of cell, Increased Targets
What are the signs, symptoms, mutations, morphology, and Hemoglobin electrophoresis of Hgb D DISEASE? SYMPTOMS: Mild hemolytic anemia, progressive splenomegaly MUTATIONS: Glycine for Glutamic Acid at position 121 ELECTROPHORESIS: Runs separate from Hgb S in citrate
What are the signs, symptoms, mutations, morphology, and Hemoglobin electrophoresis of Hgb E DISEASE? SYMPTOMS: Microcytic anemia, low MCV, target cells MUTATIONS: Lysine subbed for Glutamic Acid at position 26 on beta chain ELECTROPHORESIS: Migrates with C/A2 in alkaline and A in acid SE ASIANS
What are the 2 Apicomplexans discussed here? Plasmodium and Babesia
Plasmodium is spread by ___________ and found in tropical areas like __________ and ________. Mosquitoes, South America, Africa
________ has >15 merozoites in the schizont, has ring forms everywhere, and causes malarial complications like massive Hemolysis, cerebral malaria, and even death. Plasmodium falciparum
________ has >15 merozoites in the schizont, "shuffner's Dots", Reticulocytes are larger. They enter through the ________, so it is NOT present in Africa. Plasmodium vivax, Duffy Antigen
Bebesia is spread by _______ and found throughout the _________. It can also be spread by blood transfusion and cause systemic disease. Arthropod vectors, United States
What are the 6 Hemoglobinopathies? Trait vs. Disease, Sickle Cell Disease, Hgb C, Hgb SC, Hgb D, and Hgb E
What is Unstable Hemoglobin Disease? They are unstable Hgb variants with an INCREASED O2 AFFINITY. They have no clinical impact besides Congenital HEINS BODY ANEMIA and Congenital NONSPHEROCYTIC HEMOLYTIC ANEMIA.
________ is Autosomal Dominant where all patients are heterozygous, and 25% of patients are anemic. In the lab, you find ________ and __________. Before splenectomy Hgb is 7-12g/dL and Retics are 4-20%, and after Hgb is higher retics stay high. Unstable Hemoglobin Diseas, Heinz bodies, Basophilic stippling,
What is the difference between Cellulose Acetate gels and Citrate gels?
Why are there different affinities needed for O2? Certain conditions call for it, such as Increase in acid, temperature, pCO2, or 2,3BPG
If the shift is to the RIGHT, than means Hgb is _________ O2 more easily. Conditions where pO2 IS HIGHER. (DECREASED AFFINITY) Giving up
If the shift is to the LEFT, _________ O2 is needed. (INCREASED AFFINITY) Less
Why does the fetus need to have a greater affinity for O2 than adults do? (It shifts to the left of the O2 dissociation curve.) The placenta is not well-established before 10 weeks
In Hemoglobins THROUGHOUT LIFE, ______ is produced first then alpha, _____ is produced first then gamma, and _________&__________ come after birth. Zeta, Epsilon, Beta&Delta
____ starts early and is part of the fetal Hgb, and ______ starts after birth. Alpha, Beta
For ALPHA THALESSEMIA, _____ mutants can have 2,3,4 mutations and ______ mutants can have 1,2 mutations. Cis, Trans
What happens if there are 4 mutations in Alpha Thalessemia? Hydrops fetalis. Edema in subcutaneous tissues, Severe anemia (microcytic, hypochromic, nRBCs), Hgb Barts
What happens if there are 3 mutations in Alpha Thalessemia? Hgb H disease, mild/moderate chronic Hemolytic Anemia, Hgb 7-10g/dL, Retics 5-10%, ERYTHROID HYPERPLASIA, SPLENOMEGALY. Peripheral smear= POIKYLOCYTOSIS (target cells), Brilliant Cresyl blue shows ppt Hgb H (RASPBERRY)
What happens if there are 2 mutations in Alpha Thalassemia? Mild anemia, MICROCYTIC HYPOCHROMIC RBCs, Hgb BARTS present at birth, not present in adults bc they make enough Beta chains to prevent Hgb H formation
What happens if there is 1 mutation in Alpha Thalessemia? Silent carrier, no hematologic abnormalities
What are the 3 types of MUTATIONS IN BETA THALASSEMIA? Knockouts, Frameshifts/protein mutations, Reduced production
How is Beta thalessemia detected? Gamma chain production falls off and beta chain rises
The two knockouts of Beta thal are ________ and severe transfusion dependent ________. Beta thal major, hemolytic anemia
__________ has 3-4g/dL Hgb, marrow in skull, and bone shapes are changed as the marrow infultrates ("frontal bossing") Beta Thalessemia MAJOR
Beta Thal Major can be cured by ________. Bone marrow transplantation
____________ has two minor or one severe mutation in globin chain production levels. It does not require transfusions and patient have severe anemia. Iron overload! Beta thal INTERMEDIA
Beta Thal Intermedia patients can experience Iron overload if enough Hemolysis occurs even in the ______ of transfusion. Absence
__________ has one knockout or one mutant with a moderate decrease in production levels. Hgb is 10-13g/dL, and has higher Hgb A2 and HgbF. Peripheral smear = POIKYLOCYTOSIS (_____ and ________) and STIPPLING Beta Thal MINOR, Target cells, Elliptocytes
Most _________ get sickle cell disease, most _________ get Hb H disease, and both get Beta Thal syndrome. Blacks, Asians
What are the life expectancies of each Beta thal diseases? Major = 30's Intermediate = Minor =
Why do African people lack the Duffy antigen? They are relatively resistant to invasion by P. vivax.
What are the 3 plasmodium species and how can you tell them apart? Plasmodium falciparum (banana shaped gametocytes, deaths), Plasmodium vivax (Shuffner's dots, duffy antigen), Babesia (systemic disease)
_____ is located mainly along the equator, _____ is located in Africa, _____ is located in Western Africa, _____ is located in SE Asia, ______ is located in SE Asia, ______ is in Europe. Thalassemia, HbS, HbC, Ovalocytosis, HbE, Pk deficiency
Why would hemoglobinopathies be beneficial? They help avoid endemic diseases such as malaria
Why do larger animals need a greater affinity for O2 than smaller animals? Bc smaller animals have a higher metabolic rate of O2 consumption than larger ones.
Apart from death, fetuses also undergo ______, _______, _________, and _________ when they have 4 mutations on A-thal. Hepatosplenomegaly, cardiomegaly, marked erythroid hyperplasia, extramedullary erythropoiesis
What is the consequence of having either a cis or a trans mutation in thalassemia? Cis mutation are more severe.
What are symptoms of B-Thal MAJOR? transfusion dependent hemolytic anemia, microcytic hypochromic cells Stippled cells, nRBCs, LOW MCV, Wrinkled/folded cells with clumps of Hgb Repeated transfusions = iron overload. BM transplantation = cure
Why does B-Thal MINOR have iron overload if enough Hemolysis occurs?? Fe is absorbed out of the plasma during erythropoiesis, produced cells have limited survival, and GI tract is signaled to absorb more Fe even as Fe accum. in the tissues.
Why are globin gene mutations so common in the geographic regions where they are found? They vary by regulatory elemens, RNA processing, Nonsense codons, and Frameshifts
Created by: Hamncheese52