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Hematology Exam 3
Hemoglobinopathies, Thalassemias, Parasitic Infections of RBCs, Lec 8
| Term | Definition |
|---|---|
| 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 |
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Hamncheese52
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