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Hematology Flashcards

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1
Q

What symptoms should you expect with the following Hematocrit:

25-30%

20-25%

<20%

A

25-30% = Dyspnea and fatigue

20-25% = Lightheadedness, angina

<20% = Syncope and chest pain

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2
Q

Causes of low MCV

A

Iron deficiency

Thalassemia

Sideroblastic anemia

Anemia of chronic disease

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3
Q

What is the only microcytic anemia to have an elevated reticulocyte count?

A

alpha thalassemia with 3 genes deleted

all other microcytic anemias have a low reticulocyte count

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4
Q

Cause of high MCV

A

B12 and folate deficiency

Sideroblastic anemia

Alcoholism

Liver disease or hypothyroidism

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5
Q

Macrocytic anemias all give what type of reticulocyte count?

A

Low

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6
Q

At what hematocrit do you transfuse a patient?

A

If the patient is symptomatic (SOB, lightheaded/confused, hypotensive/tachycardic, chest pain) = transfuse

If the hematocrit is very low (25-30) in an elderly patient or one with heart disease = transfuse

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7
Q

Each unit of PRBCs should raise the hematocrit by?

A

3 points per unit

Will raise the Hgb by 1 per unit

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8
Q

When is FFP NOT the answer?

A

NOT a choice for hemophilia A or B or von Willebrand disease

Used to replace clotting factors (elevated PT, aPTT, or INR)

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9
Q

Cryoprecipitate is used to replace

A

fibrinogen

Has some utility in DIC (high levels of factor VII and VWF)

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10
Q

When do you give platelets?

A

To a bleeding patient when platelet count is <50,000

CONTRAINDICATED for TTP and HUS

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11
Q

Where is iron absorbed in the body?

A

Duodenum (4 mg/day)

  • Body needs roughly 1-2 mg/day*
  • Menstruating women need 2-3 mg/day*
  • Pregnant women need 5-6 mg/day*
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12
Q

What is hepcidin?

A

Regulates iron absorption

Hepcidin levels are low in anemia of chronic disease

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13
Q

What is the most likely diagnosis for anemia with:

  1. Blood loss
  2. Menstruation
  3. Cancer
  4. RA
  5. Alcoholic
  6. Asymptomatic
A
  1. Iron deficiency
  2. Iron deficiency
  3. Chronic disease
  4. Chronic disease
  5. Sideroblastic
  6. Thalassemia
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14
Q

Target cells are seen most commonly with

A

Thalassemia

However, they can be seen with all causes of microcytic anemia

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15
Q

Anemia with iron studies showing:

  1. Low ferritin
  2. High iron
  3. Normal iron
A
  1. Iron deficiency
  2. Sideroblastic anemia
  3. Thalassemia
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16
Q

TIBC in Iron deficiency vs Chronic disease

A

Low TIBC = chronic disease

High TIBC = iron deficiency

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17
Q

Most accurate test for:

Iron deficiency

Sideroblastic anemia

Thalassemia

A

Iron deficiency = bone marrow biopsy for stainable iron which is decreased (rarely done)

Sideroblastic anemia = prussian blue staining for ringed sideroblasts

Thalassemia = hemoglobin electrophoresis (exception = alpha thalassemia, which is diagnosed by DNA analysis)

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18
Q

Most appropriate next step when MCV is elevated

A

Peripheral blood smear

Only B12 and folate deficiency (and antimetabolite medications) cause hypersegmentation

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19
Q

Pancreatic insufficiency causes what type of anemia

A

Macrocytic (need pancreatic enzymes to remove B12 from the R-protein so it can bind with IF)

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20
Q

When the vignette suggests B12 deficiency, but the B12 level is equivocal, what is the next step?

A

MMA level (only elevated with B12 deficiency)

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21
Q

What is a complication of rapid B12 or folate replacement?

A

Hypokalemia

There is no other condition in which cells are generated so rapidly that they use all the potassium

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22
Q

Bilirubin gallstones

Osteomyelitis

Retinopathy

Stroke

Skin ulcers

Avascular necrosis

A

Common manifestation of SCD

Children present with dactylitis (inflammation of fingers)

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23
Q

The best initial test for SCD?

A

Peripheral smear

Sickel cell trait (AS disease) does NOT give sickled cells

The most accurate test is hemoglobin electrophoresis

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24
Q

What lowers mortality in SCD?

A

Hydroxyurea (prevents recurrences by increasing Hgb F)

Antibiotics (ceftriaxone, levofloxacin, or moxifloxacin) with fever

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25
Exchange transfusion for SCD is used if there is severe vasoocclusive crisis presenting with:
Acute chest syndrome Priapism Stroke Visual disturbance from retinal infarction
26
Best initial test for a patient with SCD with a decreasing hematocrit?
Reticulocyte count (first clue to parvovirus) Patients with SCD have very high reticulocyte counts because of chronic compensated hemolysis. Parvovirus B19 causes an aplastic anemia crisis which freezes the growth of the marrow. Most accurate test for parvo is a PCR for DNA. IVIG = best initial therapy
27
What is the only manifestation of sickle cell trait?
Defect in the ability to concentrate the urine (isosthenuria)
28
Most accurate test for Hereditary Spherocytosis
Osmotic fragility Defect in cytoskeleton leading to abnormal round shape and loss of flexibility
29
Treatment for Hereditary Spherocytosis
Chronic **folic acid** replacement **Splenectomy** to stop hemolysis
30
Treatment of Warm Hemolysis vs Cold Agglutinin Disease
Warm = glucocorticoids, then splenectomy, then IVIG, then rituximab Cold = stay warm, rituximab (steroids and splenectomy DO NOT WORK)
31
Cyclophosphamide Cyclosporine Azathioprine Mycophenolate mofetil
Alternative treatments to diminish the need for steroids
32
Heinz bodies and bite cells
Seen in G6PD deficiency
33
Both TTP and HUS are characterized by:
Intravascular hemolysis with fragmented red cells (schistocytes) Thrombocytopenia Renal insufficiency *TTP is also associated with neurological disorders and fever*
34
What is paroxysmal nocturnal hemoglobinuria?
A clonal stem cell defect with increased sensitivity of red cells to complement in acidosis leading to aplastic anemia, myelodysplasia, or acute leukemia Deficiency of CD 55 and 59 (decay accelerating factor)
35
Most common cause of death from PNH
Thrombosis Mesenteric and hepatic veins are the most common sites
36
Define aplastic anemia
Pancytopenia of unclear etiology Acts as an autoimmune disorder in which the T cells attack the patient's own marrow
37
Treatment for aplastic anemia
allogenic bone marrow transplantation (BMT) if the patient is over 50 or there is no matched donor, the treatment is antithymocyte globulin (ATG) and cyclosporine
38
Define polycythemia vera
Unregulated overproduction of all 3 cell lines, but red cell overproduction is the most prominent Mutation in the JAK2 protein (red cells grow wildly despite a low EPO level)
39
Hypertension Heptosplenomegaly Bleeding and Thrombosis Pruritis following warm showers
Polycythemia vera *Up to 40% of patients with PV have gout*
40
Most accurate test for PV
JAK2 mutation Also look for elevated hematocrit (\>60), low EPO, and elevated B12
41
Treatment for PV
Phlebotomy and ASA to prevent thrombosis Hydroxyurea helps lower the cell count Allopurinol or rasburicase protects against uric acid rise Antihistamines *Target Hct is \< 45%*
42
Apart from PV, what is a JAK2 mutation also associated with?
Essential thrombocytosis
43
What is myelofibrosis?
Disease of older persons with a pancytopenia associated with a bone marrow showing marked fibrosis; \*blood production shifts to the spleen and liver (enlarged) Teardrop-shaped cells and nucleated RBCs \*Extramedullary hematopoiesis
44
Lab values indicative of hemolysis for normocytic anemia
Increased LDH Decreased haptoglobin Increased Tbili *Not seen in hemorrhage (exception = production problem + hemolysis)*
45
Why is iron increased in sideroblastic anemia?
Iron gets stuck in the mitochondria; cells cannot use the iron, so the body keeps sending more that also gets stuck
46
Reversible and Irreversible causes of sideroblastic anemia
Reversible = drugs, EtOH, and lead Irreversible = B6 deficiency (cannot just replace) and MDS
47
What will the hemoglobin electrophoresis show for alpha thalassemia?
Nothing (i.e., it will be normal)
48
What must you consider when treating thalassemia?
Chronic transfusion = iron overload Managed with deferasirox or deferiprone (PO iron chelators) Deferoxamine is a parenteral version phlebotomy is NEVER the answer
49
IgA attacks parietal cells
Pernicious anemia (leads to B12 deficiency d/t lack of IF)
50
How long is B12 and folate stored in the body?
B12 = 3-10 years (from animal products) Folate = 3-6 weeks (from leafy greens)
51
Is the peripheral neuropathy from B12 deficiency reversible with replenishment?
No
52
What must you consider when replacing B12?
Whether to give IM or PO Absorption problem (pernicious anemia, crohn's, or gastric bypass) = IM B12 deficient patients = PO Schilling's test helps distinguish between the two
53
Why is cow's milk problematic in infants \<1 or when consumed excessively before the age of 2?
Low in iron Also causes occult intestinal blood loss Result = **Iron deficiency anemia**
54
The most frequently tested type of acute leukemia
M3 or acute promyelocytic leukemia this is because it is associated with DIC
55
Best initial test when suspecting leukemia
Blood smear (showing blasts) Most accurate test = flow cytometry
56
Myeloperoxidase positive
AML
57
What translocation is associated with M3?
15 and 17
58
What are Auer rods and what are they associated with?
Eosinophilic inclusions associated with AML M3 is the most common associaton
59
Tdt positive
ALL Also cALLa positive
60
Unique treatment for ALL and M3
ALL = add intrathecal methotrexate to prevent relapse in the CNS M3 = add all-trans-retinoic acid (ATRA) Both AML and ALL are treated with chemo to remove blasts and induce remission
61
BCR-ABL 9:22 translocation Philadelphia chromosome
All synonymous for CML
62
Which myeloproliferative disorder has the greatest likelihood of transforming into acute leukemia (blast crisis)?
CML If untreated, 20% of patients per year
63
Treatment for CML
Translocation of 9:22 results in _overlyactive tyrosine kinase_ Tryosine kinase inhibitors = **imatinib**, dasatinib, or nilotinib *Only a **BMT** can cure CML, but this is never the initial therapy*
64
What is the most important first step in acute leukostasis reaction?
Leukapheresis (removing the excessive white cells)
65
Define myelodysplastic syndrome
MDS is a preleukemic disorder presenting in older patients with a pancytopenia despite hypercellular bone marrow
66
5q deletion
characteristic abnormality of MDS 5q deletion has an excellent response to **lenalidomide** Also look for increased MCV, nucleated red cells, and ringed sideroblasts
67
Pelger-Huet cell
most distinct lab abnormality in MDS (hyposegmented nucleus in WBC)
68
Treatment for CLL
Most people die with it, not from it Stage III (anemia) and Stage IV (thrombocytopenia) are treated with **fludarabine**, **cyclophosphamide**, and **rituximab**
69
Massive splenomegaly "Dry" tap
Hairy Cell Leukemia A smear will show hairy cells; flow cytometry will show CD11c Treat with cladribine or pentostatin
70
Best next step when patient presents with: Painless lymphadenopathy "B" symptoms
B symptoms = fever, wt loss, drenching night sweats **Next step = excisional biopsy** This is characteristic of NHL and HD *Most common wrong answer is needle aspiration*
71
How to stage NHL?
CXR, if negative then CT chest/abd/pelvis, if negative then BM Bx Stage 1 = 1 lymph node group Stage 2 = 2 or more on the same side of the diaphragm Stage 3 = both sides of the diaphragm Stage 4 = widespread disease
72
How to treat advanced NHL (stage III or IV)?
R-CHOP ****_R_**ituximab** (antibody against CD20) ****_C_**yclophosphamide** **_H_**ydroxydaunorubicin (aka **adriamycin or doxorubicin**) **_O_**ncovin (aka **vincristine**) ****_P_**rednisone** Note: Unlike HD, NHL presents in advanced stages in 80-90% of cases
73
Reed-Sternberg cells
aka lacunar histiocytes Synonymous for Hodgkin Disease
74
Pel-Epstein (cyclical) fevers Alcohol = painful lymphadenopathy
HD
75
Burkitt's lymphoma Extranodal disease
NHL
76
Treatment of HD
Stage I and II (80-90%) = local radiation with chemo Stage III and IV = ABVD **_A_**driamycin (doxorubicin) **_B_**leomycin **_V_**inblastine **_D_**acarbazine
77
When is adriamycin (doxorubicin) contraindicated?
Adriamycin is cardiotoxic Cannot use it when EF is \< 50% (best determined with MUGA or nuclear ventriculogram which is more accurate than an echo)
78
Adverse Effects of: Doxorubicin Vincristine Cyclophosphamide Cisplatin
Chemo-man Doxo = cardiomyopathy Vin = peripheral neuropathy Cyclo = hemorrhagic cystitis Cisplatin = renal and ototoxicity
79
Most common presentation for multiple myeloma?
Bone pain from pathologic fracture (bone breaks under what would be considered normal use)
80
Most common cause of death in myeloma?
Renal failure and infection RF d/t accumulation of immunoglobulins and Bence-Jones protein in the kidney (also 2/2 hypercalcemia and hyperuricemia)
81
Anion gap in myeloma?
Decreased anion gap IgG is cationic (will increase chloride and bicarbonate levels)
82
SPEP for myeloma
IgG (60%) IgA (25%) Although these are all "M" spikes, remember IgM is a separate disease called Waldenstrom macroglobulinemia
83
What is the single most accurate test for myeloma?
Bone marrow biopsy showing \> 10% plasma cells The most common wrong answer is SPEP (99% of people with an "M-spike" do NOT have myeloma)
84
MGUS
IgG or IgA spikes on an SPEP without myeloma 1% a year transform into myeloma
85
Best initial therapy for Waldenstrom Macroglobulinemia
Plasmapheresis IgM spike results in hyperviscosity
86
What is ITP
Diagnosis of exclusion Look for isolated thrombocytopenia, normal-sized spleen, increased megakaryocytes, or antiplatelet antibodies Treat with prednisone
87
Before splenectomy, what vaccinations must you give?
N meningitidis H influenza Pneumococcus
88
Best therapy for VWD?
DDAVP (desmopressin), which releases subendothelial stores of VWF Look for bleeding related to platelets that is markedly worse after taking aspirin
89
Increased PT and aPTT Low platelet count Elevated d-dimer and fibrin split products Decreased fibrinogen
Characteristic findings for DIC
90
How to confirm HIT
ELISA for **platelet factor 4 (PF4) antibodies** or the **serotonin release assay**
91
Treatment for HIT
Stop all heparin-containing products Administer direct thrombin inhibitors: **argatroban**, lepirudin, bivalirudin, and fondaparinux
92
2 main antiphospholipid syndromes
Both cause thrombosis 1. Lupus anticoagulant (associated with elevated aPTT) 2. Anticardiolipin (associated with multiple spontaneous abortions)
93
Only cause of thrombophilia with an abnormality in the aPTT
APL syndrome Requires lifelong anticoagulation Best initial test = mixing study (**aPTT will remain elevated** even after the mix) *The anticoagulation effect only exists **in vitro**; in vivo (i.e., patients) it results in increased clot formation NOT bleeding disorders*
94
Fever Anemia Thrombocytopenia Renal Fx Neuro Sxs
Think TTP Symptoms = "FAT-RN"
95
Causes of thrombocytopenia
"Alphabet Soup" HIT TTP DIC ITP
96
Pt \> 70 Hypercalcemia Renal Fx Anemia Bone Pain
MM *Get skeletal survey to look for lytic lesion (caused by osteoclast activating factor - OAF), not a bone scan*
97
SPEP (+) UPEP (-) Skeletal (-) BM Bx \< 10%
MGUS
98
SPEP (+) UPEP (-) Skeletal (-) BM Bx \> 10% lymphoma (not plasma)
Waldenstrom Macroglobulinemia
99
Treatment for ITP
Steroids then IVIG then Rituximab
100
Bernard Soulier
GlyIb deficiency
101
Glanzman thromobocytopenia
GlyIIbIIIa deficiency

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