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68 Cards in this Set
- Front
- Back
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When do you see Spherocytes?
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hereditary
autoimmune hemolysis |
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When do you schistocytes?
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DIC, traumatic hemolysis
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When do you see Teardrop RBCs?
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Myeloid metaplasia with myelofibrosis
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When do you see Acanthocytes?
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Spiny appearance in abetalipoproteinemia
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When do you see Target cells?
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HALT
HbC disease Asplenia Liver Disease Thalassemia |
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When do you see Poikilocytes?
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TTP/HUS
Microvasc damage DIC |
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When do you see Burr cells?
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TTP/HUS
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What are some major causes of Iron deficiency anemia?
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blood loss- menorhagia, GI bleed
Diet- rare except in newborns, Increased requirement- preggers, growing people |
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What is plummer-vinson syndrome?
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iron deficiency with an obstruction of the upper esophageal web.
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What are the measures of serum Fe, ferritin, and TIBC in Fe deficiency, Anemia of Chronic disease, and Iron overload
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Fe, Ferritin, TIBC
Fe deficiency- low, low, high Anemia of chrnoic disease- low, high, normal Fe overload- High, High, normal |
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What are the general features of Pernicious Anemia?
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autoimmune;
gastritis w/ failur of intrinsic factor. Achlorydria- absent gastric free hydrochloric acid Anti-IF and antiparietal cell antibodies Increased incidence of gastric carcinoma |
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What are the clinical findings in Pernicious anemia?
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Insidious onset
lemmon-yellow skin stomatitis and glossitis Subacute degen of the spinal cord. demylenation of the posterior and lateral columns - ataxic gait, hyperreflexia w/ extensor plantar reflexes and imparied position and vibration sensation. |
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What labs can be seen in Pernicious anemia?
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Pancytopenia, hypersegged neutrophils, megaloblastic hyperplasia
Anti-IF Abs Abnl Schilling |
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What are the causes of B12 deficiency?
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Pernicious Anemia
Total gastric resection Disorders of the distal ileum- absorbed in distal iluem Strict veg diet Intestinal malabsorption Blind-loop- bacterial overgrowth- surgically induced blind loop Diphyllobothrium latum- fish tapeworm |
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What are teh causes of Folate deficiency?
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Dietary- Chronic alcoholics, fad dieters
Pregnancy- additonal demands Dilantin or OC therapy Folic acid antagonist chemo Relative deficiency- ncreased demand Intestinal malabsorptioin- sprue, giardia |
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Anemia of chronic disease has what kind of RBCs?
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normochromic and normocytic
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What are some causes of Aplastic Anemia?
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Rads
benzene Chloramphenicol Alkylating agents antimetabs Parvovirus EBV HIV Fanconi's Idiopathic Acute hepatitis |
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What are the pathologic features of Aplastic Anemia?
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pancytopenia; normal cell morph; hypocellular BM w/ fatty infiltration.
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What is the Rx for Aplastic Anemia?
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W/drawal of offending agen, allogenic BM transplant, RBC and platelet transfusion, G-CSF or GM-CSF.
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What are the Sx of aplastic anemia?
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Fatigue, malaise, pallor, purpura, mucosal bleeding, petechiae, infection
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What are the general features of Hemolytic anemias?
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Increased RBC destruction- Increased bili-> gallstones, jaundice, urine urobilinogen; Hemoglobinemia, hemoglobinuria
Hemosiderodosis No serum haptoglobins Increased Epo- Normoblastic erythroid hyperplasia in BM. Reticulocytosis Polychromatophillia |
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What is Warm Ab autoimmune hemolytic anemia?
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Most common form of hemolytic anemia.
mediated by IgG 2ry to SLE or lymphomas - spherocytosis - + Coombs |
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What are teh features of Cold agglutinin disease?
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IgM abs
Acute- I blood group Ag Mono or Mycoplasma infection Chronic- lymphoid neoplasms anti-i Abs agglutination and hemolysis in tissue sites exposed to the cold |
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What is erythroblastosis fetalis?
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maternal alloimmunization against fetal- Rh or ABO
can cause hydrops fetalis kernicterus- develops from the increased unconjugated bilirubin |
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What is paroxysmal nocturnal hemoblobinuria?
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intracorpuscular defect- aquired
increased sensitivity to complement- intravasc hemolytic anemia comes from PIG-A mutation-> decreased glycosylphosphatidlyinsoitol (GPI) Increased urine hemosiderin CD 55,59 |
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What are some trates of hereditary spherocytosis?
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Splenomegaly
osmotic fragility increase in mean corpuscular hemoglobin concentration. |
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What are the molecular defects in hereditary spherocytosis?
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spectrin, ankyrin, protein 4.1
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G6PD deficiency:
what is the inheritance? What is it? What can trigger it? what is a bite cell? |
X-linked- mediterraneaan and aftrican
self-limited intravasc hemolytic anemia w/ hemoglobinemia and hemglobinuria Some triggers- pirmaquine, slufonamides, oxidant drugs, fava beans bite cell, looks like a piece was muched out. can be found on a peripheral smear |
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What is the genetic mutation in HbS?
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codon 6 point mutation on the B-globin gene;
no recognition site ofr the restriction endonuclease MstII. Crew cut on an xray from increased epoiesis |
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What are the major complications for a Sickler?
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Severe anemia
chronic leg ulcers vaso-occlusive pain crises (infection, cold, dehydration) Repeated infarction of spleen and lung Aplastic crisis- fall in hemoglobin- parvovirus Infection- salmonella osteomyelitis |
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What is Hemoglobin C disorder?
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African
mild hemolytic anemia prominent splenomegaly target cells intraerythrocytic crystals |
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What are the Hemoglobin E disorders?
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SE asia
similare to Hemoglobin C |
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What are some new Rx for sickle cell?
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hydroxyurea-> increase HbF
BM transplant |
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What are the characteristics of B-thal major?
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Mediterranean or Cooley anemia
Anemia- decrease in Hb synth, Shortening of RBC life span, agg of insoluble alphas, ineffective epo, folate deficiency Splenomegaly Distortion of skull, facial bones and long bones Thal red cell morph- hypochromia, target cells Increased HbF Hemosiderosis- hemolysis |
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WHat are the characteristics of B-thal minor?
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minimal hypochromic microcytic anemia.
increase in HbA2 |
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What are the characteristics of the alpha-Thals?
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SE Asia
Deletions of one or more of the four alpha-globin genes Barts- gamma4 (all 4 are gone), H- gamma4 (3 gone) |
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How does scurvy manifest as a primary homeostasis disorder?
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has gingival hemorrhages, bleeding into muscles and sub cutaneous tissue. Cuutaneous petechiae- about hair follicles
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What is the mechanism of Henoch-Schonlein purpura
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Hemorrhagic urticaria- fever arthralgias, and GI and renal involvement.
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What is hereditary hemorrhagic telangiectasia?
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AD- localized malformations of venules and capillaries of the skin. Complicated by hemorrhage.
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How does Ehlers'Danlos syndrome contribute to 1ry hemostasis?
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inherated- abnlties of collagen and elastin can manifest in vascular bleeding, articular hypermobility, dermal hyperelasticity, and tissue fragility
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What is waldenstrom macroglobulinemia?
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Vasc damage from sludging of hyperviscous blood.
Can cause platelet functional abnlties |
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What are two infections that hurt 1ry hemostasis by affecting blood vessels?
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Rocky Mountain spotted fever and meningococcemia
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What is myelophthisis?
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decreased productin of platelets due to bone marrow replacement by tumor cells
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What major chronic diseases causes a 2nry thrombocytopenia?
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AIDS, SLE
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ITP- what is the difference between kids and adults?
is it a threat to the fetus? how do you Dx? |
kids- acute, self-limiting reaction to viral infection or immunization
adults- chronic disorder Maternal IgG can cause fetal thrombocytopenia. Dx- increased megakaryocytes, Ab |
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What is seen in the labs of DIC?
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schistocytes, increase in fibrin split products
Increased PT, PTT, decreased platelets, |
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What are the clinical characteristics of TTP?
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Hyaline microthrombi in small vessels, thrombocytopenia, microangiopathic hemolytic anemia
- schistocytes and helmet cells Transient neuro abnlties, renal insufficiency, fever |
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What is bernard-soulier disease?
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AR disorder with large platelets and lact of glycoprotein GPIb-IX
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What is the pathologic mechanism of DIC?
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QActivation of the coag cascade leading to microthrombi and global consumption of platelets, fibrin and coag factors
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What are some causes of DIC?
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Obstetric complications
G- sepsis Transfusion Trauma Malig Acute pancreatitis nephrotic syndrome |
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what are the leading causes of vit k deficiency?
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fat malabsorption
incomplete intesital colonization of Vit-K bacteria |
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What does PT measure?
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II, V, X, VII
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What are the Vit K clotting factors?
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II, VII, IX, X
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What is the dual hemostatic defect of vWF defeciency?
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faliure of platelet adhesion
functional deficiency of F8. |
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What is the function of vWF?
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carrier protein of F8
mediates adhesion of platelets to subendothelium at sites of vasc injury glycoprotein GPI-IX-V |
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What is the mode of inheritance of vWF disease?
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Autosomal
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What is glanzmann's thrombasthenia?
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disorder of aggregation
Decreased GP IIb-IIIa |
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What is the mechanism of action of Heparin?
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Catalyzes activation of ATIII
Decreased thrombin and Xa. Short 1/2 life LMWH- act more on Xa, better bioavailability, 2-4x longer T/2- not as easily reversed |
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What are the SEs of heparin?
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bleeding, thrombocytopenia, drug-drug interactions
rapid reversal of heparinization. Protamine referses |
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What is the mehcanims of action of warfarin?
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interferes with the normal synth of gamma carbox
inhibs factors II, VII, IX, X, C and S |
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What are the thrombolytics?
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sterptokinase, urokinase, tPA, APSAC (anistreplase)
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What is teh mechanismof the thrombolytics?
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directly or indirectly convert plasminogen to plasmin- cleaves thrombin and fibrin clots
used in MI, early ischemic stroke |
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what are the SEs of thrombolytics?
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bleeding, contraindicated in pts w/ active bleeding, Hx of intracranial bleeding, recent surgery, known bleeding diathesis, or severe hypertension. Rx tox with aminocaproic acid.
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What can counteract tPA and urokinase?
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Aminocaproic acid
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What are the SEs of aspirin?
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gastric ulceration, bleeding, hyperventilation, Reye's syndrome, tinnitus
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What is the mechanims of action of Clopidogrel?
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ihibits platelet aggregation by irreversibly blcoking ADP receptors. Inhibit fibrinogen binding by preventing IIb/IIIa expression
used in acute coronary syndrome, coronary stenting, decreased incidence or recurrence of thrombotic stroke |
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What is the major side effect of ticlopidine?
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Neutropenia
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What is teh mechanism of action of Abciximab?
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monoclonal Ab that binds to the glycoprotein receptor IIB/IIIA on activated platelets
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