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68 Cards in this Set

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When do you see Spherocytes?
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?
HALT

HbC disease
Asplenia
Liver Disease
Thalassemia
When do you see Poikilocytes?
TTP/HUS
Microvasc damage
DIC
When do you see Burr cells?
TTP/HUS
What are some major causes of Iron deficiency anemia?
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
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?
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?
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?
Pancytopenia, hypersegged neutrophils, megaloblastic hyperplasia

Anti-IF Abs
Abnl Schilling
What are the causes of B12 deficiency?
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?
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?
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?
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?
Splenomegaly
osmotic fragility
increase in mean corpuscular hemoglobin concentration.
What are the molecular defects in hereditary spherocytosis?
spectrin, ankyrin, protein 4.1
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?
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?
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?
African
mild hemolytic anemia
prominent splenomegaly
target cells
intraerythrocytic crystals
What are the Hemoglobin E disorders?
SE asia
similare to Hemoglobin C
What are some new Rx for sickle cell?
hydroxyurea-> increase HbF
BM transplant
What are the characteristics of B-thal major?
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?
minimal hypochromic microcytic anemia.
increase in HbA2
What are the characteristics of the alpha-Thals?
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?
schistocytes, increase in fibrin split products
Increased PT, PTT, decreased platelets,
What are the clinical characteristics of TTP?
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?
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