Heme 2
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- When do you see Spherocytes?
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hereditary
autoimmune hemolysis - When do you schistocytes?
- DIC, traumatic hemolysis
- When do you see Teardrop RBCs?
- Myeloid metaplasia with myelofibrosis
- When do you see Acanthocytes?
- Spiny appearance in abetalipoproteinemia
- When do you see Target cells?
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HALT
HbC disease
Asplenia
Liver Disease
Thalassemia - When do you see Poikilocytes?
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TTP/HUS
Microvasc damage
DIC - When do you see Burr cells?
- TTP/HUS
- 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 - What is plummer-vinson syndrome?
- iron deficiency with an obstruction of the upper esophageal web.
- 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 - 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 - 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. - What labs can be seen in Pernicious anemia?
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Pancytopenia, hypersegged neutrophils, megaloblastic hyperplasia
Anti-IF Abs
Abnl Schilling - 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 - What are teh causes of Folate deficiency?
-
Dietary- Chronic alcoholics, fad dieters
Pregnancy- additonal demands
Dilantin or OC therapy
Folic acid antagonist chemo
Relative deficiency- ncreased demand
Intestinal malabsorptioin- sprue, giardia - Anemia of chronic disease has what kind of RBCs?
- normochromic and normocytic
- What are some causes of Aplastic Anemia?
-
Rads
benzene
Chloramphenicol
Alkylating agents
antimetabs
Parvovirus
EBV
HIV
Fanconi's
Idiopathic
Acute hepatitis - What are the pathologic features of Aplastic Anemia?
- pancytopenia; normal cell morph; hypocellular BM w/ fatty infiltration.
- What is the Rx for Aplastic Anemia?
- W/drawal of offending agen, allogenic BM transplant, RBC and platelet transfusion, G-CSF or GM-CSF.
- What are the Sx of aplastic anemia?
- Fatigue, malaise, pallor, purpura, mucosal bleeding, petechiae, infection
- 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 - What is Warm Ab autoimmune hemolytic anemia?
-
Most common form of hemolytic anemia.
mediated by IgG
2ry to SLE or lymphomas
- spherocytosis
- + Coombs - 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 - 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 - What is paroxysmal nocturnal hemoblobinuria?
-
intracorpuscular defect- aquired
increased sensitivity to complement- intravasc hemolytic anemia
comes from PIG-A mutation-> decreased glycosylphosphatidlyinsoitol (GPI)
Increased urine hemosiderin
CD 55,59 - What are some trates of hereditary spherocytosis?
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Splenomegaly
osmotic fragility
increase in mean corpuscular hemoglobin concentration. - What are the molecular defects in hereditary spherocytosis?
- 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 - 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 - 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 - What is Hemoglobin C disorder?
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African
mild hemolytic anemia
prominent splenomegaly
target cells
intraerythrocytic crystals - What are the Hemoglobin E disorders?
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SE asia
similare to Hemoglobin C - What are some new Rx for sickle cell?
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hydroxyurea-> increase HbF
BM transplant - 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 - WHat are the characteristics of B-thal minor?
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minimal hypochromic microcytic anemia.
increase in HbA2 - 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) - How does scurvy manifest as a primary homeostasis disorder?
- has gingival hemorrhages, bleeding into muscles and sub cutaneous tissue. Cuutaneous petechiae- about hair follicles
- What is the mechanism of Henoch-Schonlein purpura
- Hemorrhagic urticaria- fever arthralgias, and GI and renal involvement.
- What is hereditary hemorrhagic telangiectasia?
- AD- localized malformations of venules and capillaries of the skin. Complicated by hemorrhage.
- How does Ehlers'Danlos syndrome contribute to 1ry hemostasis?
- inherated- abnlties of collagen and elastin can manifest in vascular bleeding, articular hypermobility, dermal hyperelasticity, and tissue fragility
- What is waldenstrom macroglobulinemia?
-
Vasc damage from sludging of hyperviscous blood.
Can cause platelet functional abnlties - What are two infections that hurt 1ry hemostasis by affecting blood vessels?
- Rocky Mountain spotted fever and meningococcemia
- What is myelophthisis?
- decreased productin of platelets due to bone marrow replacement by tumor cells
- What major chronic diseases causes a 2nry thrombocytopenia?
- AIDS, SLE
-
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 - What is seen in the labs of DIC?
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schistocytes, increase in fibrin split products
Increased PT, PTT, decreased platelets, - 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 - What is bernard-soulier disease?
- AR disorder with large platelets and lact of glycoprotein GPIb-IX
- What is the pathologic mechanism of DIC?
- QActivation of the coag cascade leading to microthrombi and global consumption of platelets, fibrin and coag factors
- What are some causes of DIC?
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Obstetric complications
G- sepsis
Transfusion
Trauma
Malig
Acute pancreatitis
nephrotic syndrome - what are the leading causes of vit k deficiency?
-
fat malabsorption
incomplete intesital colonization of Vit-K bacteria - What does PT measure?
- II, V, X, VII
- What are the Vit K clotting factors?
- II, VII, IX, X
- What is the dual hemostatic defect of vWF defeciency?
-
faliure of platelet adhesion
functional deficiency of F8. - What is the function of vWF?
-
carrier protein of F8
mediates adhesion of platelets to subendothelium at sites of vasc injury
glycoprotein GPI-IX-V - What is the mode of inheritance of vWF disease?
- Autosomal
- What is glanzmann's thrombasthenia?
-
disorder of aggregation
Decreased GP IIb-IIIa - What is the mechanism of action of Heparin?
-
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 - What are the SEs of heparin?
-
bleeding, thrombocytopenia, drug-drug interactions
rapid reversal of heparinization.
Protamine referses - What is the mehcanims of action of warfarin?
-
interferes with the normal synth of gamma carbox
inhibs factors II, VII, IX, X, C and S - What are the thrombolytics?
- sterptokinase, urokinase, tPA, APSAC (anistreplase)
- What is teh mechanismof the thrombolytics?
-
directly or indirectly convert plasminogen to plasmin- cleaves thrombin and fibrin clots
used in MI, early ischemic stroke - what are the SEs of thrombolytics?
- bleeding, contraindicated in pts w/ active bleeding, Hx of intracranial bleeding, recent surgery, known bleeding diathesis, or severe hypertension. Rx tox with aminocaproic acid.
- What can counteract tPA and urokinase?
- Aminocaproic acid
- What are the SEs of aspirin?
- gastric ulceration, bleeding, hyperventilation, Reye's syndrome, tinnitus
- What is the mechanims of action of Clopidogrel?
-
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 - What is the major side effect of ticlopidine?
- Neutropenia
- What is teh mechanism of action of Abciximab?
- monoclonal Ab that binds to the glycoprotein receptor IIB/IIIA on activated platelets