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PathomaSUN > Ch4 Hemostasis > Flashcards

Ch4 Hemostasis Flashcards

1
Q

Polycythemia vera

A

M: RBC, JAK2 mutation
E: could be reactive polycythemia due to hypoxemia or ectopic EPO production
T: phlebotomy, hydroxyurea

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2
Q

Folate/B12

A

M: THF donates methyl group to B12 which donates so homocysteine can become methionine, B12 also converts methymalonic acid to become succinyl CoA
E: poor diet, pregnancy/cancer, hemolytic anemia, methotrexate administered
T: administration, but giving folate in B12 may make symptoms initially worse

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3
Q

essential thrombocythemia

A

M: platelets, JAK2 kinase

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4
Q

follicular lymphoma

A

M: CD20 positive B cells form follicle like nodules, driven by t(14;18), over expressino of Bcl2,
T: rituximab

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5
Q

MM

A

M: proliferation of plasma cells in bone marrow, high IL-6, growth factor for plasma cells. M spike due to IgG and IgA increase
E: most common primary malignancy of bone

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6
Q

Microangiopathic hemolytic anemia (MAHA)

A

M: speed bumps/platelet microthrombi in small vessels causing shearing of RBC. Schistocytes
E: TTP and HUS- TTP is due to decreased ADAMTS13, which cleaves vWF into monomers; defect leads to abnormal platelet adhesion. HUS is due to endothelial damage by E coli Verotoxin
T: Plasmapheresis and corticosteroids

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7
Q

Heparin induced thrombocytopenia (HIT)

A

M: platelet destruction because of binding to PF4 (platelet factor), fragments of destroyed platelets activates other platelets, causes thrombosis
E: just given heparin
T: stop heparin. Don’t give warfarin

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8
Q

Vitamin K deficiency

A

M: Vit K deficiency, activated by epoxide reductase, carboxylates coag factors
E: newborns (gut flora not mature), long term abx (gut flora destroyed), malabsorption of fat soluble viamins
T: Give Vit K

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9
Q

Hairy cell leukemia

A

M: neoplastic proliferation of mature B cells with hairy cytoplasmic processes positive for TRAP (tartrate-resistant acid phosphatase)
T: 2-CDA (cladribine) an ADA inhibitor, adenosine accumulates to toxic levels in cancerous B cells

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10
Q

ATIII deficiency

A

Anti-thrombin III is activated by Heparin like molecules (or heparin) and is made by endothelial cells, so deficiency means heparin doesn’t work properly, PTT does not rise accordingly. Must give high dose of heparin

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11
Q

MGUS

A

M: preMM, increased serum protein with M spike on SPEP (serum protein elctrophoresis)
E: common in elderly, 1% develop MM each year

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12
Q

sickle cell anemia (SCA)

A

M: autosomal recessive mutation in B chain of hemoglobin- glutamic acid changed to valine, Hb sickles when deoxygenated, presenting with extra/intravascular hemolysis and extramedullary hematopoeisis
E: infants- dactylitis, unvaccinated- encapsulated organism due to autosplenecotmy, adults- acute chest syndrome, sickle cell trait is not symptomatic
T: hydroxyurea- increases HbF

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13
Q

autoimmune hemolytic anemia (AIHA or IHA)

A

M: antibody mediated destruction of RBCs. Warm (extravascular)- IgG (can cause spherocytosis), Cold (intravascular)- IgM. ca
E: for warm- SLE, CLL, drugs. For cold- mycoplasma or EBV infections
T: coombe’s test direct (blood) and indirect (serum) IVIG, steroids, stop drug

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14
Q

disorders of fibrinolysis

A

M: overactive of plasmin or underactive antiplasmin that leads to excessive cleavage of serum fibrinogen, NO DDIMER
E: old uncles with prostatectomy (urokinase activates plasmin), cirrhosis reduces production of antiplasmin
T: aminocaproic acid which blocks activation of plasminogen

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15
Q

Glucose-6-phosphate-dehydrogenase deficiency

A

M: x-linked recessive disorder with reduced half-life of G6PD, which leads to oxidative stress–> heinz bodies and bite cells. Glutathione, which is antioxidant, is produced by NADPH, a byproduct of G6PD.
E: african, mediterranean (worse variant), drugs (sulfa,, primaquine, dapsone) and fava bean eaters
T: remove and avoid trigger

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16
Q

Hemophilia B aka

A

Christmas!

M: Factor IX (refer to hemo a)

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17
Q

thalassemia

A

M: decreased synthesis of globin chain due to gene deletion (alpha thal) or gene mutation (beta thal)
E: Asians (cis deletion) african (trans deletion)
T: iron chelation, splenectomy, repeated transfusions

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18
Q

burkitt lymphoma

A

M: CD20 positive B cells with EBV, cmyc, t(8;14)
E: young child or teen, african form involves

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19
Q

excess homocysteine

A

M: B12 or folate deficiency can lead to this, or CBS (cystathionine beta synthase) deficiency can also, leading to build up of homocysteine, can damage endothelial cells

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20
Q

Immune thrombocytopenic purpura (ITP)

A

M: IgG autoantibodies against platelet antigens
E: children after infection/immunization, or young women who have SLE
T: Corticosteroids, IVIG, splenectomy

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21
Q

mono

A

M: EBV infection that causes lymphocytic leukocytosis of CD8 positive T cells, causes LAD and splenomegaly. monospot test detects the IgM antibodies that cross-react with horse or sheep RBC (heterophile)
E: kissing teenagers
T: no contact sports, supportive

22
Q

extra vs intravascular hemolysis

A

extra- reticuloendothelial cell destruction in the spleen, intra- hemoglobinemia and -uria, hemosiderinuria, haptoglobin decreases

23
Q

Waldenstrom Macroglobulinemia

A

M: B-cell lymphoma with monoclonal IgM production, causes serum hyperviscosity
T: plasmapheresis to remove IgM

24
Q

sideroblastic anemia

A

M: defective protoporphyrin synthesis because ALAS/ALAD/ferrocheletase or B6 deficiency because of Isoniazid, causes Fe to build up in mitochondria, leading to ringed sideroblasts
E: alcohlics, TB patients, lead poisoning

25
Q

Hemophilia A

A

M: Genetic factor VIII deficiency; X-linked recessive, increased PTT
E: males in royal family! but can arise denovo
T: recombinant FVIII

26
Q

Bernard-Soulier syndrome and Glanzmann thrombasthenia

A

M: Gp1b and GpIIa/IIIa deficiency respectively

27
Q

Coagulation factor inhibitor

A

M: acquired ab against coagulation factor
E: FVIII most common
T: PTT does not correct upon mixing normal plasma due to inhibitor, but it does in Hemophilia A

28
Q

Hemoglobin C

A

M: HbC has LyCine, glutamic acid changed to lysine, HbC crystals on blood smear

29
Q

marginal zone lymphoma

A

M: CD20 positive B cells that expand marginal zone, a/w chronic inflammatino such as hashimotos, sjogren, hpylori. MALToma is marginal zone lymphoma in mucosal sites

30
Q

amniotic fluid embolus

A

M: enters maternal circulation during labor or delivery, it contains tissue thromboplastin, squmous cells and keratin debris and activates coagulation and thrombosis, symptoms of DIC

31
Q

Mycosis fungoides

A

M: neoplastic proliferation of mature CD4 T cells that infiltrate skin, epidermis- pautrier microabscesses. Sezary cells- nucleus looks like brain

32
Q

vWD

A

M: genetic vWF deficiency, Autosomal dominant,
E: bleeding from mucosa
T: Desmopressin stimulates release of vWF from W-P bodies

33
Q

diffuse large B cell lymphoma

A

M: CD20 B cells
E: most common form of NHL, aggressive, presents in late adulthood

34
Q

CLL

A

M: proliferation of naive B cells that co-express CD5 and CD20
E: most common leukemia, can transform to diffuse large B-cell lymphoma, marked clinically by enlarging LN or spleen

35
Q

Paroxysmal Nocturnal Hemoglobinuria (PNH)

A

M: in myeloid stem cells, lack of GPI (glycolipid anchoring protein) means no CD55 which is DAF (decay accelerating factor) which prevents complement (c3 convertase) from destroying blood cells. Happens during night in respiratory acidosis because it activates complement.
E: main cause of death is thrombosis of hepatic, cerebral, portal veins due to pathologic platelet degranulation and activation
T: eculizumab

36
Q

ALL

A

M: lymphoblasts positive for TdT (DNA polymerase) and express CD19, CD10, CD20 that accumulate in bone marrow
E: Down syndrome (after 5 years), t(12,21) in children, t(9;22) in adults (philadelphia ALL). T-ALL: in teenagers with dysphagia

37
Q

protein c or s deficiency

A

M: usually inactivates factors V and VIII
E: severe risk for warfarin skin necrosis

38
Q

CML

A

M: granulocytes and basophils, t(9;22) philadelphia chromosome, BCR-ABL fusion-tyrosine kinase
E: can transform to AML or ALL

39
Q

AML

A

M: myeloblasts positive for MPO (auer rods when crystalized), accumulate in bone marrow. Several types: acute promyelocytic leukemia (RAR –> DIC), acute monocytic leukemia and acute megakaryoblastic leukemia
E: usually in older adults, acute megakaryoblastic leukemia in down syndrome before age 5

40
Q

mantle cell lymphoma

A

M: CD20 positive B cells in mantle zone, driven by t(11;14), cyclin D1 promotes G1 to S transition
E: late adulthood with painless LAD

41
Q

Primary Hemostasis steps (4)

A
  1. Vasoconstriction- reflex neural and endothelin release from endothelial cells
  2. Platelet adhesion- vWF binds SEC, platelets bind vWF using Gp1b receptor
  3. Platelet degranulation- adhesion changes shape and degranulation with release of ADP and TXA2 (COX)
  4. Platelet aggregation- GPIIb/IIIa used to aggregate, fibrinogen in between
42
Q

anemia of chronic disease

A

M: chronic inflammation releases hepcidin from liver, which sequesters iron by limiting iron transfer from macrophages and suppressing EPO
E: chronic inflammation patients
T: treat underlying cause

43
Q

hereditary spherocytosis

A

M: inherited defect of RBC cytoskeleton-membrane tethering proteins such as ankyrin, spectrin, or band 3. membrane blebs form and them break off, leading to spherocytes, increased RDW, Howell-Jolly bodies. increased osmotic fragility in hypotonic solution- no wiggle room
T: Splenectomy

44
Q

factor V leiden

A

mutation of factor V that lacks cleavage site for deactivation by protein C and S

45
Q

hodgkin lymphoma

A

M: RS cells, Large B cells positive for CD15, CD30, secrete cytokines which cause B sxs, attract reactive WBC,may lead to fibrosis
E: nodular sclerosis subtype common in young adult female, lymphocyte depleted is most aggressive seen in elderly and HIV

46
Q

IDA

A

M: Fe absorbed in enterocytes in duodenum via ferroportin and bound to transferrin in the blood, stored in ferritin in macrophages and liver. Dietary lack and blood loss are common causes of IDA. Fe2 gets inTWO the body, acid keeps it that way
E: infants, children, elderly with ColonCA, malnutrition, malabsorption, gastrectomy
T: supplemental iron

47
Q

DIVC

A

M: pathologic activation of coag cascade, resulting in widespread microthrombi and ischemia and infarction. and comsumption of platelets and factors results in bleeding from IV sites and mucosal surfaces. LEADS TO thrombocytopenia and DDIMER (fibrin split product)
E: Amniotic fluid (contains TT) activates PT coagulation, Ecoli or Neisseria menigitidis sepsis from endotoxins, adenocarcinoma, APL (promyelocytic leuemia), rattlesnake bite.
T: underlying cause, transfuse

48
Q

myelofibrosis

A

M: megakaryocytes, JAK2 kinase, megak cells produce excess platelet-derived growth factor (PDGF) causing marrow fibrosis- tear drop cells, affects other myeloid cells

49
Q

Langerhans cell histiocytosis

A

M: proliferatino of langerhans cells (dendritic cells found in skin) birbeck granule-tennis racket on EM, CD1a and S-100 positive

50
Q

prothrombin 20210A

A

point mutation in prothrombin that results in increased gene exprsssion- leading to increased thrombin, and thrombosis!

51
Q

Adult T-cell leukemia/lymphoma

A

M: neoplastic proliferation of mature CD4 T cells, a/w HTLV-1, presents like MM but with rash
E: Japan and carribean

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