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[2b] A- step 2 CK > Med - Heme/Onc > Flashcards

Med - Heme/Onc Flashcards

1
Q

Dx test for Iron Deficiency Anemia and why

A

FerriTin < 15; measures iron storage and is specifically for IDA

Iron,TIBC,Transferrin can all be low in Anemia of Chronic Dz as well

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2
Q

Causes of Normocytic Anemia

NON-Hemolytic (normal or low Retic count) -5

vs

Hemolytic (INC Retic count) - 10

A
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3
Q

Is Haptoglobin ⬆︎ or ⬇︎ in Hemolytic Anemia? Why?

A

DECREASED; Liberated Hgb (after RBC hemolysis) BINDS to serum Haptoglobin –> HgbHaptoglobin complex –> Cleared by Liver

Haptoglobin picks up Haphazard hgb

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4
Q

Lab markers for Hemolytic Anemia -5

A
  1. ⬇︎Haptoglobin
  2. ⬆︎LDH
  3. ⬆︎UnConjugated bilirubin
  4. Hgbnuria (Reddish brown urine)
  5. DAT (Direct Antiglobulin Coombs Test) -detects antibody-mediated hemolysis
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5
Q

3 main sources of iron deficiency

A
  1. ⬇︎ Intake (PO vs GI absorption)
  2. ⬆︎Output (menorrhagia/hematuria/hemorrhage)
  3. ⬆︎ Requirement (pregnancy)
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6
Q

Most common causes of [Normocytic anemia w/HIGH RDW]-4

A
  1. Iron deficiency
  2. B12 Deficiency
  3. Beta Thalassemia
  4. hypOthyroidism
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7
Q

High RDW typically indicates what etx -4

A
  1. Mixed deficiency
  2. Recent Hemorrhage
  3. Iron deficiency Anemia (late - microcytic)
  4. Vit B12/Folate deficiency (Macrocytic)
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8
Q

[Anemia with Normal RDW] typically indicates ____

A

Thalassemia

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9
Q

What about FerriTin makes it non-guaranteed lab value for iron changes?

A

FerriTin is also an acute phase reactant and ⬆︎ with any stress

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10
Q

Spinal Cord Compression can be from DJD, Epidural Abscess or Tumor

Which Cancer metastasis are associated with Tumor Spinal Cord Compression? - 5

A
  1. Prostate
  2. Renal
  3. Lung
  4. Breast
  5. Multiple Myeloma
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11
Q

Which has a longer onset of action: Ibuprofen or Naproxen?

A

Naproxen

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12
Q

T or F: Brain Metastasis from NonSOLC is Chemosensitive

A

FALSE!

NonSOLC is NONSensitive

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13
Q

CP of Acute Intermittent Porphyria - 3

A

AIP causes PAN

  1. Psychosis acute onset
  2. Abd pain acute onset
  3. Neuropathy acute onset

​Fam hx of this is VERY suggestive of AIP

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14
Q

Acute Intermittent Porphyria dx ; What factor of a pts hx suggest

A

⬆︎Urine Porphobilinogen ; Fam hx of similar sx

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15
Q

When is Cryoprecipitated used?

A

to replace fibrinogen (DIC) - replaces clotting factors in smaller plasma

this is never used first

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16
Q

low Ferritin is specific for what type of anemia

A

iron deficiency anemia

Remember that FerriTin is an acute phase reactant

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17
Q

What does TIBC measure

A

unbound iron sites on transferrin

i.e. will be elevated in IDA but low in Anemia of Chronic Disease

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18
Q

isolated ELEVATED IRON is specific to what type of anemia?

A

Sideroblastic anemia

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19
Q

Anemia with normal iron studies is specific for what type of anemia?

A

Thalassemia (except 3 gene deletion alpha thalassemia)

Dx = Hgb electrophoresis with genetic studies if alpha thalassemia

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20
Q

Tx for iron deficiency anemia? ; sideroblastic anemia

A

ferrous sulfate 2+ ; Pyridoxine B6

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21
Q

What is Cooley Anemia? ; tx?

A

Beta thalassemia MAJOR (BOTH Beta proteins on chromo 11 are underproduced) –> SEVERE ANEMIA

Tx = chronic transfusion with deferadirox iron chelator

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22
Q

Causes of Vitamin B12 deficiency - 6

A
  1. PERNICIOUS ANEMIA = MOST COMMON CAUSE
  2. Vegan/Vegetarian
  3. Blind loop syndrome (Gastrectomy or RYGB)
  4. Diphyllobothrium latum
  5. Pancreatic Insufficiency
  6. Terminal iLeum damage (Crohns)
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23
Q

how do you differentiate Vitamin B12 deficiency from Folate deficiency

A

Vitamin B12 isomerizes methymalonyl coA in the spinal cord myelin —> succinyl coA.

Without it –> suBACute combined degeneration

usually manifest as peripheral neuropathy

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24
Q

Causes of Folate deficiency - 4

A
  1. Goat Milk diet (has no folate)
  2. Psoriasis
  3. Phenytoin
  4. Sulfa drugs
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25
Q

Chronic hemolysis is associated with what type of gallstones?

A

Pigmented bilirubin gallstones

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26
Q

In sickle cell, what causes vasoocclusive crisis -4

A

DICK

  1. Dehydration
  2. Infection
  3. Cold temp
  4. Kant breathe (HYPOXIA)
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27
Q

Sickle Cell Disease dx

A

Initially: Peripheral Smear (since SCtrait won’t have sickled cells)

Confirmation: Hgb Electrophoresis

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28
Q

What type of figures are found on smear in Sickle Cell Disease

A

Howell Jolly Bodies (left over nuclear material in pts who don’t have a spleen to remove it)

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29
Q

What disease do you see Bite cells?

A

G6PD deficiency

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30
Q

What disease do you see Morulae on peripheral blood smear?

A

Ehrlichia infection

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31
Q

Tx for Sickle Cell Pain Crisis - 4

A
  1. HOA (Hydration/Oxygen/Analgesia)
  2. Ceftriaxone if fever/WBC present
  3. Hydroxyurea ⬆︎HbF
  4. Transfusion if Acute Chest Syndrome, Priapism, Stroke, Retinal infarction
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32
Q

How does ParvoB19 affect Sickle Cell Disease pts

A

parvoB19 causes Aplastic Crisis which freezes any further reticulocyte development from marrow. SC pts usually always have high reticuloctye counts but when hit with parvoB19, they suddenly have a drop

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33
Q

Tx for Hereditary Spherocytosis - 2

A
  1. Folic acid chronically
  2. Splenectomy (stops hemolysis)
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34
Q

MOD for Warm IgG hemolysis

A

Autoimmune Ab in the spleen or liver bind to RBC and remove small amounts of the membrane –> microspherocytes

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35
Q

Tx for Warm IgG Hemolysis - 4

A

1st = ** CTS initially ** –> IVIG

2nd = Splenectomy —> Riuximab

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36
Q

What are the triggers of Cold IgM hemolysis - 3

A

cold weather is MMMiserable

  1. Mononucleosis EBV
  2. Mycoplasma PNA
  3. Macroglobulinemia Waldenstrom

occurs in colder (peripheral) parts of body and resolve with warming up body

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37
Q

Which tx for Warm IgG hemolysis can NOT be given to Cold IgM hemolysis - 2

A

CTS

SPLENECTOMY

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38
Q

Do NOT confuse Cryoglobulins with Cold IgM hemolysis

What are Cryoglobulins associated with? - 3

A
  1. Hep C
  2. Joint Pain
  3. Glomerulonephritis
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39
Q

List the 4 main characteristics of G6PD deficiency

A

Stress makes me eat bites of fava beans with Heinz ketchup

  1. oxidant Stress on RBC from lack of glutahione reductase –> hemolysis and SUDDEN BACK PAIN
  2. Bite cells
  3. Induced by fava beans, macrobid, sulfa, primaquine, infection
  4. Heinz bodies (RBC inclusions seen after crystal violet staining)

Almost always in Black/Mediterranean Men with sudden Anemia

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40
Q

HUS and TTP MOD

A

TTP WILL HAVE MORE NEURO SX and it’s tx = plasma exchange

otherwise, HUS and TTP present the same

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41
Q

TTP is associated with what conditions? - 4

A
  1. Clopidogrel
  2. Ticlopidine
  3. Cyclosporine
  4. AIDS

Unlike HUS, TTP will have neuro sx (confusion/seizures) in addition to Fever

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42
Q

When is it ok to give Platelet transfusion to HUS/TTP?

A

NEVER!!!

TTP may receive Plasma exchange for tx

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43
Q

MOD for Paroxysmal Nocturnal Hemoglobinuria

A

[CD55/59 Decay Accelerating factor] (responsible for deactivating complement) of the RBC, WBC and platelets is produced less

so…

nocturnal respiratory acidosis (shallow breathing during sleep) –> complement activation –> complement binds to RBC, WBC and platelets –> nocturnal hgbnuria/⬆︎infection/thrombosis

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44
Q

What is the most common cause of death in Paroxymal Nocturnal Hemoglobinuria

A

Thrombosis (Mesenteric or Hepatic veins)

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45
Q

Tx for Paroxysmal Nocturnal Hemoglobinuria

A
  1. CTS prednisone
  2. Bone Marrow Transplant = cure
  3. Eculizumab inactivates C5 complement
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46
Q

Treatment for Aplastic Anemia is Bone Marrow Transplant

At what age does this become unavailable? ; what are the alternatives then?-2

A

>50 yo ; [AntiThymocyte Globulin and Cyclosporine]

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47
Q

Pt has intense pruritus after a warm shower

What is the Dx? ; Why does this happen?

A

Polycythemia Vera ; Heat ⬆︎Basophils (rare but can become AML) –> ⬆︎Histamine release

  • You must exlude Hypoxia as a cause of ⬆︎RBC*
  • Dx = JAK2 mutation*
  • Remember: PV ⬆︎ALL 3 cell lines but places focus on RBC more*
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48
Q

Tx for Polycythemia Vera - 2

A
  1. phlebotomy
  2. hydroxyurea
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49
Q

When do you treat Essential Thrombocytosis?-2

A

ONLY when pt>60 yo and [sx or platelets are >1.5million]

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50
Q

Tx for Essential Thrombocytosis - 3

A
  1. HYDROXYUREA
  2. Anagrelide when RBC is suppressed from Hydroxyurea
  3. ASA for erythromelagia (painful red hands from ET)
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51
Q

Aplastic Anemia and Myelofibrosis both present as Pancytopenias

What are 3 discerning factors?

A
  1. Myelofibrosis occurs in older people
  2. Myelo = teardrop shaped cells from cells struggling to get out of fibrosed bone marrow
  3. Myelo tx = [Thalidomide and Lenalidomide] = bone marrow production stimulators
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52
Q

Acute Leukemia will present with signs of _____

Which acute leukemia is associated with DIC?

A

PANcytopenia ; promyelocytic M3 (chromo 1517)

Dx = smear showing blast –> flow cytometry for confirmation

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53
Q

Acute Leukemia will present with signs of _____

Which acute leukemia is associated with Auer rods (eosinophilic inclusions)?

A

PANcytopenia ; promyelocytic M3 (chromo 1517)

Dx = smear showing blast –> flow cytometry for confirmation

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54
Q

Acute Leukemia will present with signs of _____

Which acute leukemia is associated with Myeloperoxidase?

A

PANcytopenia ; Myelocytic

Dx = smear showing blast –> flow cytometry for confirmation

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55
Q

DDx for pt presenting with pancytopenia - 5

A
  1. Acute Leukemia
  2. Aplastic Anemia
  3. Myelofibrosis (dry tap and tear drop cells)
  4. Myelodysplastic Syndrome (hypercellular bone marrow with ringed sideroblast with Prussian blue )
  5. Hairy Cell Leukemia (dry tap with hypercell bone marrow)
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56
Q

Acute Leukemia will present with signs of _____

Which acute leukemia is associated with ATRA-all trans retinoic acid?

A

PANcytopenia ; promyelocytic M3 (chromo 1517)

Dx = smear showing blast –> flow cytometry for confirmation

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57
Q

Which acute leukemia is intrathecal MTX given to prevent relapse?

A

ALL is treated with intrathecal MTX to prevent CNS releapse

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58
Q

How is the [LAP-Leukocyte Alkaline Phosphatase] test used for Heme/Onc diagnostics?

A

Leukemic cells do NOT have high levels of Alkaline Phosphatase so in CML, LAP will be low

if LAP is high = leukemoid rxn (stress rxn)

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59
Q

Other than [LAP-Leukocyte Alkaline Phosphatase] test, what else can be used to diagnose CML?

A

[BCR-ABL 922] PCR

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60
Q

Out of the Myeloproliferative disorders, which has greatest potential for transformation into Acute Leukemia BLAST CRISIS?

A

CML

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61
Q

What are the initial therapies for Chronic Myelogenous Leukemia?-3 ; What is the ultimate cure?

etx: chromo 922 = BCR ABL gene

A

tx = tyrosine kinase inhibitors such as ..

  1. imatinib
  2. dasatinib
  3. nilotinib

Cure = Bone marrow transplant (NEVER the first therapy though)

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62
Q

In pt presenting with acute leukostasis reaction, what is the FIRST important thing to do for them?

A

Leukaphresis (establish dx AFTER!)

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63
Q

Myelodysplastic Syndrome is a PreLeukemic disorder

Why do most pts never actually develop AML from Myelodysplastic Syndrome?

A

They die of infection or bleeding FIRST

MDS causes pancytopenia despite hypercellular bone marrow

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64
Q

Which chromosomal abnormality is Myelodysplastic Syndrome associated with?

A

5q deletion (these pts have better pgn)

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65
Q

What disorder does Pelger Huet cells belong to?

Cell with Bilobed nucleus

A

MyeloDysplastic Syndrome

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66
Q

Describe peripheral blood smear for CLL-Chronic Lymphocytic Leukemia

A

proliferation of normal and mature (but dysfunctional) B lymphocytes with smudge cells

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67
Q

What is the Richter phenomenon

A

conversion of Chronic Lymphocytic Leukemia –> high grade lymphoma which happens in 5% of patients

CLL = mature lymphocytes and smudge cells

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68
Q

What px should be given in Chronic Lymphocytic Leukemia

A

PCP

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69
Q

Tx for Chronic Lymphocytic Leukemia - 2

A

Stage

[0: ⬆︎WBC] and [1: LAD] = no tx

[3: HepatoSplenomegaly], [4: Anemia], [5:Thrombocytopenia] = Fludarabine and Rituximab(if available)

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70
Q

Hairy Cell Leukemia dx?-2 ; Tx?

B cells with filamentous projections on smear

A

Dx = [TRAP-Tartrate Resistant Acid Phosphatase] or CD11c

Tx = Cladribine

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71
Q

Non-Hodgkin Lymphoma and Chronic Lymphocytic Leukemia both involve lymphocyte proliferation

What is the major difference

A

NHL = solid mass (lymph nodes and spleen)

CLL = Circulating liquid mass (so use flow cytometry of peripheral blood to diagnose)

72
Q

NonHodgkin Lymphoma cp - 2 ; Dx?

A
  1. painLESS LAD
  2. B sx (Fever, Night sweats, Wt Loss)

Dx = EXCISIONAL bx with staging via CT and BMbx to determine tx

THIS IS THE SAME AS HODGKIN LYMPHOMA - except HD has ReedSternberg owl cells

73
Q

Tx for NonHodgkin Lymphoma with Bsx or ≥ Stage 3 -5

A
74
Q

Tx for NonHodgkin Lymphoma stage 1A and 2A

A
75
Q

For Hodgkin Lymphoma, what are the determinants for prognosis?

A

Lymphocyte Predominant = GOOD Pgn

76
Q

Tx for Hodgkin Lymphoma with Bsx or ≥Stage 3 - 4

A
77
Q

In Heme/Onc what are the MUGA and nuclear ventriculogram used for?

A

Determine cardiotoxicity for Adriamycin/Doxorubicin tx used for Hodgkin Lymphoma

78
Q

What are the toxicities for Cisplatin and Carboplatin? - 2

A
79
Q

What are the toxicities for Vincristine?

A
80
Q

What are the toxicities for Bleomycin and Bulsulfan?

A
81
Q

What are the toxicities for Doxorubicin?

A
82
Q

What are the toxicities for Trastuzumab?

A
83
Q

What are the toxicities for CYclophosphamide?

A
84
Q

What are the toxicities for 5-FU?

A
85
Q

What are the toxicities for 6-MP?

A
86
Q

What are the toxicities for MTX?

A
87
Q

Multiple Myeloma MOD

A

abnml proliferation of plasma cells that produce useless immunoglobulin (IgG , IgA) –> [Osteoclast Activating Factor] and CRABB (hyperCalcemia, Renal failure, Anemia, Bone lytic lesions, Back pain)

When MM produces IgM = Waldenstrom Macroglobulinemia

88
Q

cp for Multiple Myeloma - 5

A

CRABB

(hyperCalcemia, Renal failure, Anemia, Bone lytic lesions, Back pain)

Hypercalcemia –> Bone lytic lesions, CONSTIPATION, Depression

Renal failure from Bence Jones proteinuria and Immunoglobulins –> DEATH

Infection –> DEATH

Osteoclast Activating Factor -> Hypercalcemia and Bone fxs

89
Q

Multiple Myeloma dx - 5

A
  1. Xray showing osteoLYTIC fx lesions from hypercalcemia
  2. Serum Protein Electrophoresis showing IgG or IgA monoclonal “M” Spike (can also be seen in MGUS)
  3. Bence Jones proteinuria via urine immunoelectrophoresis only
  4. Rouleaux on smear
  5. ***Bone marrow bx showing > 10% plasma cells*** = CONFIRMATION
90
Q

Multiple Myeloma tx - 4

A
  1. [Bortezomib proteasome inhibitor] and/or LD
  2. [LD- Lenalidomide + Dexamethoasone] –> Bone Marrow Transplant if < 70 yo
91
Q

What is the issue with seeing IgG or IgA monoclonal “M” spikes on serum protein electrophoresis?

A

This does NOT automatically mean Multiple Myeloma. This can be seen in MGUS-Monoclonal Gammopathy of Unknown Significance which = common in older pts and only transmorts to myleoma 1%/year

92
Q

Waldenstrom Macroglobulinemia MOD ; cp-5

A

Multiple Myeloma plasma cells overproduce IgM specifically –> hyperviscosity sx (HA, tinnitus)

  1. Neuropathy
  2. Engorged blood vessels
  3. HA
  4. tinnitus
  5. Raynaud Phenomenon
93
Q

Waldenstrom Macroglobulinemia tx - 3

A
  1. PLASMAPHERESIS initially
  2. Chlorambucil + Prednisone = long term OR
  3. Fludarabine + Prednisone = long term
94
Q

ITP cp - 2

A
  1. isolated thrombocytopenia +/- megakaryocytes on smear (typically after infection)
  2. Normal spleen

Strongly associated with HIV and HepC

95
Q

ITP tx - 5

A

Strongly associated with HIV and HepC

96
Q

What’s the most common inherited bleeding disorder

A

AUTO DOM Von Willebrand Disease

worst after using ASA, detected via Ristocetin cofactor assay

97
Q

tx for Von Willebrand disease - 3

A

DDAVP desmopressin –(refractory)–> Factor 8 or vWF concentrate

(DDAVP releases subendothelial stores of vWF)

98
Q

What type of bleeding is seen with Factor 11 Deficiency

A

USUALLY NO INCREASED BLEEDING

99
Q

What are the triggers for DIC - 6

A

his DIC SCABS Terribly!

  1. Sepsis
  2. CA
  3. Abruptio placenta or Amniotic fluid embolus
  4. Burns
  5. Snake bites
  6. Trauma –> tissue factor release

DIC activates primary AND secondary coagulation

100
Q

Tx for DIC - 3

A
  1. Platelets if < 50K
  2. FFP (clotting factors)
  3. Cryoprecipitate (replaces fibrinogen if FFP doesn’t work)
101
Q

Which type of clots are more common with HIT? ; dx for HIT?-2

A

Venous ; Platelet factor 4 Ab on ELISA or Serotonin release assay

102
Q

Lupus Anticoagulant dx

A

Russel Viper Venom test

103
Q

What is the most common complication for pts with Sickle Cell Trait?

A

Painless hematuria

Sickle cell is auto recessive

104
Q

What prophylaxis should pts who’ve recently had a splenectomy receive afterward?

A

Daily PO PCN x 5 years

105
Q

Bernard Soulier cp - 2

A
  1. Superficial Bleeding out of proportion to the degree of thrombocytopenia
  2. GIANT platelets

etx = absent platelet glycoprotein 1B-9-5 Receptor for von willebrand factor

106
Q

In pts with Factor defects (secondary coagulation dysfunction), what is the long term of blood sitting in their joints?

A

Hemophilic arthropathy

iron/hemosiderin desposition –> synovitis and fibrosis of that joint –> chronic worsening joint pain and swelling

107
Q

The vast majority of Head and Neck CA (i.e. submandibular uL hard non-tender LAD) are what type of CA?

A

SQC

108
Q

Why are Bisphosphonates given to CA pts? - 2

A

stabilizes bony metastatic lesions which

  1. prevents hypercalcemia of CA
  2. prevents fx
109
Q

What is the most common cause of Folate B9 deficiency in the U.S.?

A

EtOH

110
Q
A
111
Q

What are the major electrolyte changes in Tumor Lysis Syndrome - 3

A

cytotoxic chemotherapy –>

  1. ⬆︎K+ –> cardiac arrhythmias
  2. ⬆︎uric acid (give px allopurinol and IVF)
  3. ⬆︎Phosphate (will bind and ⬇︎ serum Ca+)
112
Q

GVHD-Graft Versus Host Disease is common after _____

It involves Graft/Donor __ cells attacking which 3 parts of the body?

A

Bone marrow transplant ; T ;

  1. Skin
  2. GI
  3. Liver

This occurs because Graft/Donor T-cells recognize major and minor HLA antigens of the Host

113
Q

What’s the best tx for CA-related anorexia -2

A

Megestrol progesterone analogue >>> CTS

Marijuana is only useful in HIV anorexia

114
Q

**HIGH YIELD**

When is EPO indicated for ESRD pts?

What are the side effects of EPO? - 3

A

Hgb<10 (use EPO with hct goal of 35%)

  1. HTN
  2. HA
  3. Flu-like sx
115
Q

How can you differentiate between Bone Marrow Infiltration and Bone Marrow Aplasia using the spleen?

A

Splenomegaly only occurs in Bone Marrow Infiltration

116
Q

Describe Splenic Sequestration

A

complication of Sickle Cell Disease in which a pooling of RBC AND PLATELETS within the spleen –> splenomegaly, pallor and thrombocytopenia

117
Q

Sickle Cell Disease pts are at risk of having Aplastic Crisis

What is the difference between Aplastic Crisis and Aplastic Anemia?

A

Aplastic Anemia is a/w PANcytopenia and can be congenital (fanconi, drug-induced, autoimmune)

118
Q

Sickle Cell Disease pts are at risk of having Aplastic Crisis

Clinical Presentation of this? ; What’s the most common cause of this?

A
  1. SUDDEN drop in Hgb with
  2. Reticulocyte < 1%
  3. NO Hepatosplenomegaly

Parvo B19!

119
Q

Fanconi anema is an auto recessive disorder that causes ____(micro/normo/macro) anemia

cp?-3

A

Fanconi MACROcytic anemia

  1. PANcytopenia marrow failure
  2. morphological changes
  3. growth stunt
120
Q

Laboratory results for Chronic Myelogenous Leukemia?-3

A
  1. ⬆︎ABSOLUTE BASOPHILIA
  2. ⬆︎⬆︎⬆︎LEUKOCYTOSIS
  3. shift tward precursor cells (myelocytes or promyelocytes)
  4. ⬇︎Leukocyte Alkaline Phosphatase (LAP)

Cure = Bone marrow transplant (NEVER the first therapy though)

etx = 922 BCRABL philadelphia chromosome

121
Q

Dx for Chronic Myelogenous Leukemia?-3

A

LOW Leukocyte Alkaline Phosphatase score (marker of neutrophil activity and differentiates from leukomoid rxn)

122
Q

lab values for Acute Lymphoblastic Leukemia - 3

A
  1. > 25% Lymphoblast from bone marrow bx (THIS IS HOW YOU DIAGNOSE)
  2. TdT positive (TdT is only expressed by preB and preT lymphocytes)
  3. PAS positive

cp: LAD, hepatosplenomegaly, thrombocytopenia

123
Q

DDx - 4

A

Target cells = HALT

  1. HbC
  2. Asplenia
  3. Liver disease
  4. Thalassemia (usually asx and REQUIRES NO TX if asx - occurs in Mediterranean people)
124
Q

Describe the Transfusion Reaction timeline - 4

A
125
Q

What is the px for Febrile nonhemolytic reactions after transfusion

A

Leukoreduction of donor blood

this will also ⬇︎ risk of HLA alloimmunization and CMV transmission

126
Q

Factor 5 Leiden mod ; how does this affect aPTT and PT/INR

A

AUTO DOM point mutation in Factor 5 gene –> RESISTANCE TO PROTEIN C (which is supposed to inactive Factor 5). This –> Hypercoagulability

aPTT AND PT/INR may both be normal!

127
Q

Dx for Hereditary Spherocytosis - 3

A

E5 with Acid

[Eosin 5 maleimide binding flow cytometry] WITH [Acidified glycerol lysis test]

OR

Osmotic fragility test but it has low sensitvity

Lab findings = ⬆︎Mean Corpuscular Hgb Concentration

128
Q

why are sickle cell patients (both trait and disease) at ⬆︎for benign nocturia

A

HYPOSTHENURIA ; This is when sickles obstruct the vasa rectae of the inner medulla –> inability to concentrate urine

Happens in Sickle Cell pts

129
Q

Autoimmune Hemolytic Anemia and Hereditary Spherocytosis BOTH can cause extravascular hemolytic anemia

How can you discern the two? - 2

A

Fam hx and Coombs test

AIHA = Positive Coombs test (negative fam hx)

Hereditary Spherocytosis = NEG Coombs test (positive fam hx)

130
Q

G6PD deficiency MOD

A

Stress makes me eat bites of fava beans with Heinz ketchup

Triggers –> hemolysis

Almost always in Black/Mediterranean Men with sudden Anemia

131
Q

Hereditary Spherocytosis MOD

A

Defect in RBC membrane (ankyrin, band and spectrin) –> spleen removing the defective parts lil by lil as RBC past which eventually –> spherocytes which have High mean cell hgb concentration and RBC distribution width

132
Q

In Transfusion Reactions both Anaphylaxis and Acute Hemolytic Reactions can occur within minutes

How do you differeniate the two?

A

Anaphylaxis (especially if pt is IgA deficient) = Respiratory Distress

Acute Hemolytic Reaction = Flank pain with F/C

133
Q

Which type of clots are Heparin Induced Thrombocytopenia pts at risk for, Venous or Arterial?

A

BOTH

134
Q

A pt coming in with GI distress secondary to EColi should be worked up for what heme condition

A

Hemolytic Uremic Syndrome HUS HAT

HUS HAT from EColi O157 H7 shiga toxin

Hemolytic Anemia

Acute renal failure

Thrombocytopenia

135
Q

What are the 3 examples of Microangiopathic Hemolytic Anemia

A

HAT (HemolyticAnemia/AcuteRenalFailure/Thrombocytopenia)

  1. DIC
  2. HUS
  3. TTP (will have more neuro signs)
136
Q

What do anabolic steroids affects hematologic lab values? - 3

A
  1. ⬆︎RBCs
  2. Hepatotoxic
  3. Dyslipidemia
137
Q

Dx

A

Thalassemia

image shows teardrop cells (Thalassemia also has Target cells)

138
Q

what’s the most common cause of anemia in premature infants?

A

Anemia of Prematurity

etx: ⬇︎EPO, shortened RBC life

139
Q

TTP tx

A

TTP will have more neuro sx and it’s tx = plasma exchange

140
Q

DIC tx

A

Cryoprecipitate

(contains clotting factors, fibrinogen and vWF)

141
Q

Hereditary Spherocytosis MOD

A

Defect in RBC membrane (ankyrin, band and spectrin) –> spleen removing the defective parts lil by lil as RBC past which eventually –> spherocytes eventually –> hemolysis

Triad = Splenomegaly, Jaundice, Hemolytic Anemia

142
Q

Which hematological abnormality is Acute Cholecysitis a major complication of?

A

Hereditary Spherocytosis (from pigmented gallstones)

Triad = Splenomegaly, Jaundice, Hemolytic Anemia

143
Q

Although it is a procoagulant, why is lupus anticoagulant called an anticoagulant?

A

because ONLY in the petri dish, it causes prolonged aPTT

144
Q

Describe the peripheral blood smear for Chronic Myeloid Leukemia

A

leukocytosis with shift toward precursor cells (promyelocytes, myelocytes)

Cure = Bone marrow transplant (NEVER the first therapy though)

145
Q

What is Trousseau Syndrome? ; What does it indicate?

A

hypercoagulable disorder –> recurrent migratory superficical thrombophlebitis at unusual sites (arm, chest) ;

Pancreatic Cancer (or sometimes stomach, lung or prostate)

146
Q

When is empiric anticoagulation ok for DVT/PE?

A

ONLY when pt has sx suggesting PE is present . If only DVT sx are present, confirm with US first before anticaogulation

147
Q

How long does it take a vegan to develop macrocytic anemia secondary to vitamin B12 deficiency

A

≥4 years

148
Q

A white male presents with megaloblastic anemia, atrophic glossitis, vitiligo and neuro problems…

all consistent with Vitamin B12 deficiency

What is likely the cause?

A

GENETIC! Whites of Northern European ancestry naturally develop Pernicious Anemia

also, Pernicious Anemia ⬆︎ risk for gastric ADC

149
Q

Describe etx for Warfarin induced skin necrosis

A

Warfarin ⬇︎2, 7, 9, 10, Protein C and S

27910 = ⬆︎Clotting

[Protein C and S] = anti-Clot = Bleeding. But Protein C and S are the first to be affected by warfarin, allowing 27910 to rome freely and ⬆︎Clotting

150
Q

Pernicious Anemia is the most common cause of Vitamin B12 deficiency

Pernicious Anemia ⬆︎ risk for developing what type of cancer?

A

Gastric ADC

151
Q

What is the mangement for when a pt develops Heparin Induced Thrombocytopenia (HIT) - 3

A

1st: STOP HEPARIN OR ENOXPARIN
2nd: initiate alternative (dabigatran, fondaparinux)
3rd: initiate Warfarin once platelet is > 150K

152
Q

An elderly pt has minor trauma and develops ecchymoses

What should be first on the DDx for this particular pt?

A

Senile Purpura (not heme related)

Age-related ⬇︎of elastic fibers in perivascular connective tissue –> purpura

153
Q

What disease should you suspect in a pt with Macrocytic anemia and congenital anomalies?

A

Diamond Blackfan Syndrome (DBS)

intrinsic defect in erythroid progenitor cells –> ⬆︎apoptosis

154
Q

What gene abnormality causes Chronic Myelogenous Leukemia?

A

chromo 922 which forms BCR ABL gene

Cure = Bone marrow transplant (NEVER the first therapy though)

155
Q

Type of Cell? ; Diagnosis?

A

Atypical Reactive CD8 T cells; Infectious Mononucleosis

156
Q

MOD for Hairy cell leukemia? ; How is diagnosis made?

A

B cell neoplasm that infiltrates bone marrow, spleen and peripheral blood; Bone Marrow Biopsy

157
Q

Diagnosis? ; MOD of this disease?

A

Gaucher ; lysosomal storage disease

158
Q

Describe what Transferrin measures?

A

The amount of iron being transferred in the blood

159
Q

Describe what Total Iron Binding Capacity (TIBC) measures?

A

The Capacity of Iron transferrin can actually carry

160
Q

Describe the following values for Iron Deficiency Anemia:

MCV

Iron

Transferrin

TIBC

Ferritin

A
161
Q

Describe the following values for Thalassemia:

MCV

Iron

Transferrin

TIBC

Ferritin

A
162
Q

Describe the following values for Anemia of Chronic Disease:

MCV

Iron

Transferrin

TIBC

Ferritin

A
163
Q

Describe the following values for Sideroblastic Anemia:

MCV

Iron

A

⬇︎MCV

⬆︎Iron

164
Q

How do you diagnose CLL-Chronic Lymphocytic Leukemia?

Suspect this in any elderly with dramatic leukocytosis primarily made of lymphocytes

A

Flow Cytometry of peripheral blood

proliferation of normal and mature (but dysfunctional) B lymphocytes with smudge cells

165
Q

An elderly pt who presents with dramatic leukocytosis primarily made of lymphocytes should always make you suspicious of what disease?

A

Chronic Lymphocytic Leukemia (CLL SLL)

proliferation of normal and mature (but dysfunctional) B lymphocytes with smudge cells

166
Q

MOD for Pica

A

iron deficiency anemia –> desire to eat ice, clay, dirt, paper

167
Q

A pt is newly diagnosed with Head and Neck Squamous cell carincoma

What is the next best step in their diagnostic process?

A

PANendoscopy (esophagoscopy, bronchoscopy, laryngoscopy)

168
Q

Pt has intense pruritus after a warm shower

What is the Dx? ; What is the tx for this?

A

Polycythemia Vera ; Phlebotomy

  • You must exlude Hypoxia as a cause of ⬆︎RBC*
  • Dx = JAK2 mutation*
  • Remember: PV ⬆︎ALL 3 cell lines but places focus on RBC more*
169
Q

Pt has intense pruritus after a warm shower. Polycythemia Vera is diagnosed

What is the difference between Phlebotomy and Plasma exchange?

A

Phlebotomy (tx for PV) removes cells while Plasma exchange only removes substances (Antibodies, immune complexes, toxins)

170
Q

What is the most common type of testicular sex cord stromal tumor? ; What does it secrete?-2

A

Leydig ; Testosterone AND Estrogen

All Solid Testicular Tumor Masses should be treated with Radical Orchiectomy

171
Q

A pt has been diagnosed with a Solid Testicular Tumor Mass via Ultrasound

Tx? -2

A

Radical Orchiectomy –> additional chemoradiation/surgery based on cell type

172
Q

Identify cells ; What disease are they associated with?

A

Spur cell acanthocytes ; Liver disease

173
Q

What blood disorder should be suspected in a pt with ⬆︎Mean Corpuscular Hgb Concentration?

A

Hereditary Spherocytosis

174
Q

cp for Immune Thrombocytopenia ; tx?-3

A

isolated thrombocytopenia after an infection (usually in a child) ;

  1. usually self limited to 6 months = observation
  2. IVIG if bleeding present OR
  3. CTS if bleeding present
175
Q

What type of gallstones are pts with Hereditary Spherocytosis at risk for?

A

Pigmented Bilirubin Gallstones

in Hereditary Spherotycosis, RBC membrane defect can cause intravascular hemolysis

176
Q

Triad for Osler Weber Rendu syndrome

A

Osler Weber like to EAT

  1. Epistaxis recurrently
  2. AV malformations
  3. Telancietasia
177
Q

Which 2 Vitamins are used to treat Homocystinuria?

A

Pyridoxine B6 with Folate B9

[2b] A- step 2 CK (16 decks)

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