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Heme Once Flashcards

1
Q

Primary Hemostasis

A

Injury to blood vessels causes platelet aggregation. Vasospasm, Adhesion, Activation, Aggregation

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2
Q

Vasospasm

A

Reflex neural vasospasm and also release of endothelin causes constriction of blood vessels

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3
Q

Adhesion

A

Collagen binds to vWF which is released from endothelial cells (WP bodies) and platelets (alpha granules). vWF binds to gp1b and activates platelet

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4
Q

Activation

A

Binding of Gp1b causes release of Ca and ADP from dense core granules. ADP activates the expression of gp2b3a (inhibited by clopidrogel, ticlopidine activation) which binds fibrinogen (released from alpha granules).

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5
Q

Aggregation

A

Platelets cross linked with fibrinogen and gp2b3a (gp2b3a blocked directly by abciximab). Release of COX activates TXA2 which promotes aggregation (aspirin irreveribly acetylates and deactivates COX)

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6
Q

Secondary Hemostasis

A

Clotting cascade ends with activation of factor 10 which activates factor 5 (cleaved by C, defect in factor V Lieden), which activates factor 2 (thombin) which cleaves fibrinogen to fibrin which is cross linked.
-Requires a phospholipid surface (platelets) and Ca

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7
Q

Intrinsic Pathway

A

12 to 11 to 9 (Hemophilia B) to 8 (hemophilia A, autoimmune to factor 8) to 10.
Followed with PTT
Heparin has more effect on Intrinsic Pathway

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8
Q

Extrinsic Pathway

A

7 to 10
Followed by PT (INR)
Warfarin Best Measurment

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9
Q

Kalikrein Pathway

A

HMWK cleaved by active factor 12. Leads to Kalikrein production

  • Kalikrein activates Plasminogen to plasmin which cleaves fibrin and fibrinogen and prevents platelet aggregation
  • Kalikrein also activates Bradykinin responsible for vasodilation, pain, permiability
  • Plasmin activates C3 to begin complement cascade
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10
Q

Vitamin K Factors

A

2,7,9,10,C,S.

Warfarin induced skin necrosis because C has shorter halflife. Give heparin during initial warfarin dose

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11
Q

Protein C

A

Activated by protein S and Cleaves Factor 5.

-Factor 5 Lieden

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12
Q

Endothelial Derived anticoagulant Factors

A

NO, PGI2, Thrombomodulin

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13
Q

Thrombomodulin

A

Turns thombin into protein C activator (anti coagulant)

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14
Q

Hemophilia A

A

XR, can be de novo
Defect in Factor 8. Impaired extrinsic cascade, elevated PTT normal PT and bleeding time
Will improve with mixing studies
-Bleeding into joints, easy brusing, rebleed following surgery (tooth extraction)

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15
Q

Coagulation Factor inhibitor

A

Autoimmune to Factor 8
Same presentation with bleeding into joints, increased PTT
Does not improve with mixing studies

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16
Q

Hemophilia B

A

Same presetation as A except there will be an absence of factor 9

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17
Q

ITP

A
  • Autoimmune, IgG Ab to platelet produced in spleen. Platelets removed by splenic macrophages.
  • Increased bleeding time with normal PT and PTT, -Decreased platelet count and increased megakaryocytes in BM
  • Clinically epistaxis, menorhaggia, increased skin and mucosal bleeding.
  • Acute: Most commonly in kids, manage symptomatically and with steroids, generally resolves
  • Chronic: Seen more in adult women of childbearing age, treat chronically with steroids
  • IVIG can also be used to treat.
  • IgG can be passed to fetus through placenta
  • Splenectomy is curative in refractory cases.
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18
Q

TTP (Thrombotic Thrombocytopenic Purpura)

A
  • Decreased ADAMSTS13 (most commonly autoAb to ADAMSTS13)
  • Normally ADAMSTS13 cleaves vWF multimers into monomers for rapid degredation. Loss of ADAMSTS13 prevents cleavage and leads to activtion of coagulation cascade
  • Formation of Microthrombi, Consumption of platelets, microangiopathic hemolytic anemia.
  • Increased bleeding time with normal PT and PTT
  • Also more commonly causes neurologic signs and fever. May also cause renal failure, but more common in HUS.
  • Tx: Plasmaphoresis and immunosuppressives
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19
Q

HUS

A
  • Following infection with E Coli O157:H7 (bloody diahrrea from undercooked meat)
  • Release of verotoxin which inhibits protein synthesis (23s/60s)
  • Microangiopathic hemolytic anemia at kidney
  • Also causes fever and renal failure. Less commonly causes neurologic signs (more likely TTP)
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20
Q

Bernard Soulier

A

Defect in gp1b prevents adhesion of collagen to vWF to platelets
-Large platelets with defects in primary hemostasis
Decreased platelet number and increased bleeding time
-Increased mucosal bleeding, menorrhagia and epistaxis

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21
Q

Glanzmans

A

Defect in gp2b3a prevents primary hemostasis

  • Increased bleeding time
  • Increase in bleeding time with no decrease in platelets
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22
Q

ATIII Deficency

A

Lack of ATIII which normally inhibits all aspects of the coagulation cascade, will lead to hypercoagulability
-Treatment with normal dose heparin will be ineffective, must give higher dose.

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23
Q

Uremia

A

Impairs platelet aggregation and will lead to increased bleeding times

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24
Q

Factor V Lieden

A

Very Common

Prevents cleavage by protein C which makes it more active and increases coagulability

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25
Q

Homocysteinuria

A
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26
Q

vW Disease

A
  • Most common inherited bleeding disorder
  • Decreased vWF leads to impaired platelet adhesion and also decreased factor 8 (normally vWF stabalizes)
  • Increased bleeding time and increase in PTT, normal PT. Classically will see menorrhagia
  • Abnormal Ristocetin test (normally causes vWF to bind platelets and cause activation, absence of vWF prevents this from happening)
  • Tx: Desmopressin causes release of remaining vWF from WP bodies on endothelial cells.
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27
Q

Vitamin K Deficency

A
  • Normally produced from gut bacteria and is used by epoxide reductase to gamma carboxylate factors 2,7,9,10,c,s. Creates Ca binding sites
  • Seen in newborns (prophylactic vitamin K injections)
  • Alterations of gut bacteria
  • Malabsoprtion
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28
Q

Liver failure and large volume transfusions

A

Cause impaired secondary coagulation because of decreased clotting factor concentration.

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29
Q

Tissue Thromboplastin

A
  • Glyoprotein on smooth muscle and fibroblasts that activates factor 7
  • Present in Amniotic Fluid (embolism)
  • Present in atherosclerotic plaques.
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30
Q

DIC

A
  • Innapropriate activation of clotting cascade.
  • Sepsis (LPS, IL-1, TNF)
  • Obstetric (HELLP), (tissue thromboplastin is in amniotic fluid)
  • Adenocarcinoma (mucin)
  • Rattlesnake
  • PML (Auer Rods) contain primary granules (azurophilic, proteolytic enzymes)
  • Micropangiopathic anemia, throbmocytopenia, consumption of clotting factors
  • Increased PT, PTT, Bleeding time. Decreased platelets and increased megaK in BM. D-Dimer and split products, decreased fibrinogen
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31
Q

HIT

A
  • Hapten formation with platelt factor 4 and heparin leads to autoimmune destruction of platelets
  • Increased Bleeding time
  • Destroyed platelet fragments may activate clotting cascade
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32
Q

Radical Prostatectomy

A

Release of urokinase leads to activation of plasmin and cleavage of fibrinogen. Also may be seen with malignancy.

  • Fibrin split products without D-Dimers
  • Elevation PT and PTT, destruction of clotting factors
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33
Q

Alpha 2 antiplasmin

A

Cirrhotic liver will reduce and lead to increased plasmin activation

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34
Q

Aminocaproic Acid

A

Blocks plasminogen activation. Overdose of tPA or inappropriate activation

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35
Q

Lines of Zahn

A

Alternating layers of RBC and FIbrin deposits

Seen in thrombosis

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36
Q

Disruption of Normal Blood Flow

A

A fib/heart wall motion abnormalitiy
Immobility
-Anyeurism

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37
Q

Endothelial anticoagulant factors

A
  • Heparin like molecules and ATIII destroys clotting factors
  • NO, PGI2 cause dilation and prevent aggregation
  • Secrete tPA
  • Block subendothelilal collagen and tissue thromboplastin
  • Thrombomodulin thrombin can activate protein C
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38
Q

Homocysteinuria

A
  • Genetic defect in cystathione beta synthase

- Thrombosis, retardation, lens dislocation, long fingers

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39
Q

Protein C/S deficency

A
  • Congenital hypercoagulable state

- Increased risk for warfarin skin necrosis

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40
Q

Prothrombin 20210A

A
  • Activating point mutation that leads to elevated levels of prothombin
  • Mutation is in 3’ UTR which will increase expression
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41
Q

Oral Contraceptives

A

-Estrogen increases production of coagulation factors leading to hypercoagulable state

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42
Q

Atherosclerotic Embolus

A
  • Rupture of protecting cap exposes collagen and thromboplastin which cause clot
  • Presence of cholesterol clefts
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43
Q

Fat Embolus

A
  • Trauma, bone or fate goes to lung
  • Petichiae and shortness of breath
  • Presence of fat blobs in thrombus
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44
Q

Gas

A
  • Diving leads to bends

- Caissons leads to hemorrhagic necrosis of bone

45
Q

Amniotic

A
  • Squamous cells and keratin in lungs

- Contains tissue thromboplastin

46
Q

DVT

A

-Most common popliteal, femoral, iliac (hypercoagulable state)
-Pleuritic chest pain, hemoptysis
-Most are clinically silent but may reorganize and cause pHTN
-Large cause hemorragic infarct wedge shaped
-Saddle is electrical/mechanical dissosiation
-

47
Q

Elevted ESR

A

Increase blood acute phase reactants leads to clumping and sedimentation at faster rate
-Infection, vasculitis, neoplasia

48
Q

Decreased ESR

A

-Sickle cell, CHF

49
Q

Fe Deficecny Anemia

A
  • Decreased Fe leads to decreased hemoglobin leads to microcytic and hypochromic anemia
  • Symptoms: Fatigue, Palor, Koilonychia, Pica
  • Labs: Decreased Ferretin, Fe. Increased TIBC and decreased percent saturation. Decreased retic count. Increased RDW and increased free protoporphyrin.
  • Fe metabolism regulated by duodenal enterocyte (celiac disease) and hemoportin. Absorbed as Fe 2+ (heme) maintained there by Vitamin C and Acid (Gastrectomy).
  • Transported as Fe3+ in transferrin and stored in macrophages of liver and BM in ferretin as Fe 2+
  • Commonly caused by dietery deficency. Also PUD in adult men, menorrhagia in adult women, Cancer in older men and women, and Necator hookworm in developing countries
  • Tx: Ferrous sulfate
50
Q

Plummer Vinson

A

-Severe Fe deficency anemia leads to glossitis, and esophageal webs (increaesed risk of SCC)

51
Q

Anemia of Chronic Disease

A
  • Chronic disease and inflammation increased hepcidin (caused by increased IL-6). Hepcidin causes Fe sequestration and decrease in EPO.
  • Symptoms of anemia, may begin normocytic
  • Caused by cancer, Autoimmune, etc
  • Labs: Increased ferretin, Fe, and percent saturation. Decreased TIBC. Increased FPP.
  • Tx: EPO if symptomatic in cancer
52
Q

Sideroblastic

A
  • Decrease prophyrin synthesis leads to Fe accumulation in mitochondria which form ringed sideroblasts in bone marrow.
  • Fe overloaded state may lead to symptoms of hemochomatosis
  • Decreased porphyrin because of congential defect in ALAS (XR), acquired decrease in B6 (isoniazid)(cofactor for ALAS), Lead poisoning (inhibits ferrocheletase (mitochondria and ALAD), alcohol
  • Labs: increased Fe, increase Ferretin, normal TIBC
  • Tx: Succimer for Pb kids, Dimercaperol and EDTA
53
Q

Lead Poisoning

A

-Pb causes sideroblastic anemia (ferrocheletase, ALAD)
-Also impaired resorption of rRNA leading to basophilic stippling.
-Lead lines on gums and bones
-Encephalopathy
-Abdominal Colic
-Wrist and foot drop
Tx: Succimer (oral) dimercaperol/EDTA (injected)

54
Q

Alpha Thalessemia

A
  • Mutation in alpha locus (4) leads to absent production.
  • 1 mutated is assymptomatic
  • 2 mutated is mildly symptomatic, maybe some HbH. If 2 mutated Cis (asian) risk of hydrops fetalis in baby, if 2 mutated trans (african) minimal risk
  • 3 mutated: Severe anemia with HbH (B4 tetramers) lead to hienz bodies and splenomegally.
  • 4 Mutated: in utero lethal with hydrops fetalis and Hb bartz (y4).
55
Q

Beta Thalessemia

A
  • Defect in reduced production of Beta chain, most often cause by splicing errors. Generates B+ and B0 (B0 is worse).
  • Minor is B/B+ leads to mild hypochromic microcytic anemia with few target cells. Will show mild reduction in HbA and mild increase in HbA2
  • Major is Bo/Bo leads to severe microcytic, hyopchromic anemia. Significant increaese in HbA2 and HbF. Does not present in fetus because of HbF.
  • Intermediate is somewhere between.
56
Q

Beta Thal Major

A
  • Bo/Bo Splice error
  • Major increase in HbA2 and HbF
  • Target Cells
  • Extramedullary hematopoesis and elevated EPO. Hepatosplenomegally, Crew cut skull, increased Retic
  • Alpha 4 tetramers lead to hienz bodies and splenomegally and extravascular hemolysis
  • Highly susceptible to parvovirus aplastic crisis
  • BM transplant is currative
  • Require transfusions leading to secondary hemochromatosis (Defuroxamine)
57
Q

Folate Deficency

A

Macrocytic megaloblastic anemia with hypersegmented neutrophils.

  • Caused by impaired Thymidine synthesis leads to decreased DNA synthesis
  • Dietary, Absorption (jejunum), Increaed Demand (pregnancy) Drugs (methotrexate, phenytoin, trimethoprim)
  • Normally folate gives methyl to B12 then to homocysteine to make methionine.
  • Labs show elevated homocysteine and reduced folate
  • Low body storage can develop quick
58
Q

B12 Deficency

A
  • Macrocytes with hypersegmented neutrophils
  • Elevated homocysteine and methylmalonic acid, also subacute combined degeneration.
  • Absorbed in terminal ileum with R binder and IF
  • Caused Pernicous anemia, Crohns, Pancreatic insufficency, Diphylattum
  • Rarely diet because of large hepatic stores
59
Q

Orotic Aciduria

A
  • Impaired UMP synthase leads to decreased pyrimidines
  • Megaloblastic anemia that does not correct with Folate or B12, also orotic acid in urine
  • Distinguish between OTC (XR) by absence of elevated nitrogen and decreasd BUN, also no anemia with OTC
60
Q

Nonmegaloblastic Macrocytics

A
  • Alcoholism
  • Liver Disease
  • Drugs 5-FU, 6MP, Hydroxyurea (HbF)
61
Q

Extravascular hemolysis labs

A

-Increased UCB, LDH

62
Q

Intravascular Hemolysis

A

-Decreased Haptoglobin, increased LDH, hemoglobin in urine

63
Q

Aplastic Anemia

A

Radiation
Drugs (Chlorpromazine, Chloremphenicol, alkylating etc)
Virus (Herpes family, parvo)
Fanconi’s anemia (DNA repair defect)
-Pancytopenia with fatty replacement of marrow
-Tx: Marrow stimulators

64
Q

CKD

A

Decrease EPO leads to anemia

65
Q

Spherocytosis

A
  • Defect in ankyrin or spectrin or band3 leads to membrane fragility and blebs removed by spleen.
  • Normocytic anemia with spherocytes (lack central palor), Increased RDW and MCHC.
  • Spleen consumes RBC that are misformed and splenectomy will cure anemia but spherocytes will still be present.
  • Specific diagnoisis by osmotic fragiltiy
  • Risk of aplastic crisis with parvovirus
  • Elevated UCB and increased risk for pigmented galstones
66
Q

Sickle Cell disease

A
  • Point mutation in globin chain with a G to V switch. Causes B chains to be at risk for sickling under hypoxic, acidotic, dehydrated conditions.
  • Infarcted autosplenectomy due to vasocclusion is small and firbotic. Increased risk of encapsulated infection and is most common cause of death in children with sickle cell.
  • Risk for salmonella osteomylitis (can survive in Macrophages)
  • Vasocclusive crisis causes dactylitis in kids fingers and toes and is often presenting symptom
  • Acute chest syndrome is vasoclussive crsis involving lungs and is most common cause of death in older sickle cell patients.
  • Can also cause renal papillary necrosis with hematuria and proteinuria
  • Increased risk for aplastic crisis (parvovirus)
  • Electrophoresis will show HbS
  • Treatment with hydroxyurea will increase HbF production which is protective
  • Extramedullary hematopoesis with hepatomegally and crew cut appearance of skull
  • Splenic sequestration is trapping of RBC in spleen leading to rapid drop in RBC and may cause shock
67
Q

Sickle Cell Trait

A

Heterozygotes of sickle cell gene

  • 60% HbA with 40% HbS show minimal symptoms but often show renal damage with hematuria over time.
  • Metabisulfate can cause sickling in both disease and trait
68
Q

HbC

A
  • G to Lysine transition
  • Extravascular hemolysis and cells will show classic crystals
  • If with HbS will be milder form of sickle cell
69
Q

G6PD

A

XR decerase in the half life of G6PD. Leads to decresed NADPH in RBC (No nucleus to replace G6PD.

  • African variant will have longer half life and is less severe than the mediterranian variant.
  • NADPH required to replenish reduced glutathione in response to oxidative stress
  • Oxidative stress: Drugs (Sulfa and anti-malarial), Infection, Fava beans
  • Oxidative stress will cause Hb denaturation and the production of Hienz bodies leading to bite cells
  • Intrvascular and Extravascular hemolysis, back pain, red urine, decreased haptoglobin, jaundice.
  • Diagnose with enzyme test, only reliable after the episode has resolved.
70
Q

PK Deficency

A

AR defect in PK, cells that rely on glycolysis exclusively are more effected.

  • RBC has decreased ATP leading to impaired membrane integriy and hemolysis
  • Second most common cause, often presents in newborn with hemolysis and jaundice
71
Q

Paroxysmal Nocturnal Hemoglobinuria

A

-Acquired defect in GPI anchor prevents DAF and MERL from being present on surface of blood cells and platelets.
-At night mild respiratory acidosis leads to complement activation and pure intravascular hemolysis.
-Hemoglobin in urine, esophageal spasm
-Most severe complication from lysed platelet particles that cause thrombus formation. DVT, PE, Budd-Chiari
-Absence of CD55 (DAF) on flow cytometry
-10% of patients will develop AML
Tx: Ecluzimab (complement inhibitor)

72
Q

Cold Agglutanins

A
  • IgM Ab that are directed against RBC. Can agglutinate and activated complement in cool extremeties and fingers.
  • Causes intravascular hemolysis
  • Caused by Mycoplasma Pneumonia and Mono. Also CLL.
  • Direct Coombs will be positive
73
Q

Warm Agglutanins

A
  • IgG to RBC and often hapten. Secreted by spleen most of the time, extravascular hemolysis by splenic macrophages.
  • Commonly caused by drugs (penicilin/cephalosporins, methyl-dopa), CLL, SLE
  • Direct Coombs Positive
74
Q

Coombs Test

A

Direct Coombs uses IgG to Ig portion to detect Ab bound to RBC
-Indirect uses patients serum to detect Ab present to RBC (Blood typing, hemolytic disease newborn)

75
Q

Microangiopathic

A

Intravascular and extravascular hemolysis with schistocytes

  • TTP (ADAMSTS13), HUS (E coli O157:H7), DIC
  • HELLP (Microangiopathic during pregnancy at liver)
76
Q

Macroangiopathic

A

RBC destruction with schistocytes and helmet cells from prosthetic heart valves or aortic stenosis

77
Q

Malaria

A
  • Faliprum Causes cerebral malaria and severe disease with vaiable (generally 24hr) hemolysis
  • Vivax/Ovale - 48hr fever with hypnozites in liver
  • Malaria: 72 hr
  • Chloroquine is first line treatment. Mefloquine if resistant, quinidine if severe.
  • Primaquine for Vivax/ovale to take care of liver hypnozites
  • Drugs often cause hemolytic anemia
  • Anoepheles mosquito
78
Q

Babesia

A
  • Ixodes tick Commonly co infects with lyme disease
  • Maltese cross inclusions with hemolytic anemia
  • More severe in patients who are asplenic
79
Q

Estrogen (Pregnancy or OC) effects on Iron Studies

A
  • Cause increase in Fe binding proteins.
  • Decreased Saturation and increased TIBC
  • Also will cause hemodilution and physiologic anemia of pregnancy
80
Q

Porphyria Cutanea Tarde

A
  • Mutation in Uroporphyrinogen decarboxylase leading to accumulated uroporphilinogen. (Cytoplasm)
  • Induced by drugs that increase oxidative stress (catalase) or induce P-450s (alcohol and barbituates are contraindicated.
  • Presents with blistering and photosensitvity, tea colored urine
  • Tx: Dextrose and Heme prevents de novo synthesis
81
Q

Acute Intermitent Porphyria

A
  • Defect in porphobilinogen deaminase (cytoplasm) leads to accumulation of porphobilinogen and other non uro precursors
  • Patients will have psychiatric, GI, and red urine
  • Incited by drugs, barbituates and alcohol are contraindicated.
  • Tx: Dextrose and heme to decrease de novo synthesis
82
Q

Leukemoid reaction vs Leukemia

A
  • Leukemoid is physiologic and will have increased Leukocyte Alkaline phosphatase (LAP)
  • Leukemia will not have increased LAP and will show increase in basophils in some cases (CML)
  • Monocytosis indicates chronic infection or cancer
83
Q

Hodgkins Lymphoma

A
  • Reed Sternberg cells are CD15/30 positive and are binucleate, owl eye, large cells.
  • Secrete cytokines that will call in all kinds of inflammatory cells, bulk of tumor is inflammatory cells, not RS cells.
  • Will present with single or small group of LN generally in the neck/mediastinum. Often also present with constitutional B symptoms (less likely in NHL)
  • Most common subtype is nodular sclerosing with bands of fibrous tissue. Also lymphocyte count is determinant with lower lymphocyte count being poor prognosis
  • Bimodal distribution with young and old
84
Q

Non Hodgkins Lymphoma

A
  • Multiple subtypes
  • Nodal involvment is often more extensive then HL
  • Rarely presents with constitutional symptoms
85
Q

Follicular

A
  • Small cell and common. B cells arranged in follicles with destruction of overall architecture and absence of macrophages.
  • t(14:18) IgHc and BCL-2 renders cells antiapoptotic
  • May transform into DLBLC (single enlarging LN)
  • Generally is hard to treat and has an indolent course
86
Q

LN Structure

A
  • Cortex has follicles and is B cell area
  • Paracortex has T cells
  • Medulla has sinuses and histiocytes, often site of cancer mets
87
Q

Mantle Zone Lymphoma

A
  • NHL with predominance of cells just outside follice
  • CD5+
  • t(11:14) puts cyclin D on IgHc promotor, increased progession from G1 to S
88
Q

Marginal Zone

A
  • NHL, normally no marginal zone but presence in lymphoid hyperplasia
  • Not associated with translocation but arises in chronic inflammation
  • Hashimotos, Sjogrenns, H Pylori
89
Q

Burkitt’s Lymphoma

A
  • Intermediate size cells, often caused by EBV
  • African variety in Jaw and Sporadic variety in abdomen
  • t(8:14) places C-myc oncogene on IgHc promotor
  • Will show massive cellular growth with starry sky appearance of blue cells. Macrophages will still be present.
90
Q

DLBCL

A
  • Large cells and aggressive, worse prognosis of all kinds
  • May arise in the context of CLL/SLL or from follicular NHL
  • 20% of tumors are T cell in origin
91
Q

Adult T-Cell Lymphoma/Leukemia

A
  • Caused by HTLV-1 (lentivirus) most commonly effects asian, western africa, and carribean
  • Commonly involves skin and often presents in Neck
  • Will have lytic bone lesions with hypercalcemia (use skin involvment and absence of LC to distinguish between Multiple Myeloma)
92
Q

Mycosis Fungoides/Sezeary Syndrome

A
  • Cutaneous T cell lymphoma
  • If gets into blood is called Sezeary syndrome, lymphocytes will be large and show cerebriform nuclei.
  • Usually CD4+
93
Q

Multiple Myeloma

A
  • Leukemia of mature B cells, can be caused from a variety of mutations generally involving Hc (14) and can arise from MGUS
  • Driven by cytokines especially IL-6
  • Presentation with bone pain that shows lytic lesions and hypercalcemia. Will also show anemia.
  • M spike will be visible in the gamma globulin fraction
  • Excess light chains will be secreted leading to AL amyloid and myeloma kidney
  • Excess LC will also cause loss of zeta potential and roleaux formation
  • Infection is the most common cause of death, especially from encapsulated organisms.
  • On smear/biopsy there will be plasma cells with clock face nucleus
  • Tx: Thalidomide, Bisphosphonates, chemo, and definitive cure with BM transplant
94
Q

AL amyoloid and bence jones proteins

A
  • Most commonly and strikingly effect heart and kidney. Can arise from Waldenstroms or Multiple Myeloma
  • Kidney will show proteinuria and edema with presence of Bence Jones proteins in urine
  • Heart will show deposits that may lead to constrictive cardiomyopathy and arrythmias
  • Can also cause hypervisosity leading to headache and blurry vision
95
Q

Waldenstroms Macroglobulinemia

A
  • Multiple Myeloma that secretes IgM and does not have lytic bone lesions.
  • Will still show AL amyloid
96
Q

MGUS

A
  • M spike and oversecretion of monoclonal Ab that is not associated with other signs of Multiple Myeloma
  • May progress to multiple myeloma ate rate of 1%/yr
97
Q

B-ALL

A
  • Lymphocytic Leukemia with more than 20% blasts in BM (Makes acute) tdt positive
  • CALLA+
  • CD 10, 19, 20 are markers
  • Increased risk in trisomy 21
  • t(9:22) worse prognosis, also t(12:21) better
  • Most common cause of death is infection from all leukemia and lymphomas
  • Tx: Prophylactic chemo to testes and brain because of BBB (same for T-ALL)
98
Q

T-ALL

A
  • Lymphocytic greater than 20% blasts in marrow, tdt positive
  • Markers are CD3-8
  • CALLA+
99
Q

CLL/SLL

A
  • Naive B cells, CD5 and CD20+
  • Older patients with slow indolent course. Less than 20% blasts in marrow. CLL is in blood and SLL is in Marrow
  • May transition to acute or DLBCL
  • Smudge cells will be present on biopsy
  • Associated with warm hemolytic anemia (tx with steroids)
  • Often hypogammaglobulinemia or monoclonal gamma globulin
100
Q

Hairy Cell

A
  • Mature B cells with cytoplasmic hairy extensions
  • TRAP positive
  • Splenomegally of RED PULP with dry tap on BM aspiration
  • No Lymphadenopathy
  • Tx: Cladribine which is a ADA inhibitor (Adenosine analog) adenosine builds up to toxic levels and causes cell death
101
Q

AML

A
  • Greater than 20% blasts on BM biopsy. Many subtypes
  • MPO positive, rupture or treatment may induce DIC
  • May rise in the background of myelodysplastic syndromes such as CML
  • Most common cause of death is infection, will also show anemia
102
Q

APL

A

Subtype of AML with t(15:17) RAR. Cuases produciton of immature B cells

  • Presence of auer rods.
  • Treated with ATRA which will cause maturation of immature cells and resolution
103
Q

Monocytic

A

-Infiltrates gums

104
Q

Megakaryocytic

A
  • Commonly seen in downs patients less than 5 years

- Lacks MPO

105
Q

CML

A
  • Myelodysplasia, presence of t(9:22) philadelphia chormosome. BCR-ABLE
  • Distinuish from leukemoid with low LAP and large amounts of basophils
  • Also often presents with splenomegally
  • Have pancytosis with Myeloid lineage most
  • May accelerate to AML (2/3) or ALL (1/3) mutation is in HSC
  • Tx: is TK inhibitor imatinib
106
Q

Myeloproliferative disorders

A
  • Pancytosis that progresses to marrow fibrosis and burnout
  • Increased risk for gout with nucleus turnover.
  • Can transform to AML/ALL
107
Q

Polycythemia Vera

A
  • Increase in Circulating RBC Driven by JAK2 Mutation
  • Presents with hyperviscosity (Headache and blurred vision), Thrombosis (Budd-Chiari and others), Itching (elevated mast cells)
  • Can also be secondary to other conditions, distinguish
  • Concentration from loss of plasma volume
  • EPO production (RCC, HCC, Hydronephrosis, Wilms tumor, VHL)
  • Physiologic response to cardio/respiratory problems, or high altitude/smoking.
  • Tx: Phlebotomy and hydroxyurea
108
Q

Essential Thrombocytosis

A
  • Increased platelets in circulation, often driven by JAK mutation
  • MegaK present in marrow and release platelet fragments
  • Rarely progresses to leukemia because of lack of nucleus in periphery
109
Q

Myelofibrosis

A
  • End stage of myelodysplasia with fatty replacement of BM
  • Driven by PDGF and other fibrotic signals
  • Anemia with teardrop cells
  • Extramedullary hematopoesis leading to splenomegally
  • Nucleated RBC in periphery (loss of structure and reticulin gates)

Boards Path (20 decks)

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