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Hematology (4%) Flashcards

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1
Q

Acute Lymphocytic Leukemia (ALL)

A

Most common childhood cancer (ALL kids have cancer)
ALL = Anemia, lumps, limp
Bone marrow biopsy = > 20% lymphoblasts
CNS prophylaxis with intrathecal methotrexate

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2
Q

Acute Myeloid Leukemia (AML)

A

Middle aged
Acute Promyelocytic Leukemia (APL) = most common subtype
Bone marrow biopsy = > 20% myeloblasts

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3
Q

Acute Promyelocytic Leukemia (APL)

A 
Auer rods (A-rod plays in the big APL)
Treatment = chemo PLUS Vitamin A
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4
Q

Chronic Lymphocytic Leukemia (CLL)

A 
Older adults (70 yr)
Smudge cells (CLL --> Crushed Little Lymphocytes)
IF asymptomatic --> observe
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5
Q

Chronic Myeloid Leukemia (CML)

A

Phl chromosome = 9 –> 22 translocation
Treatment = tyrosine kinase inhibitors (Imatinib)

Tyrone is a country music lover (CML) and went to concert in Philadelphia on 9-22.

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6
Q

Thrombotic Thrombocytopenic Purpura (TTP)

A 
Severe deficiency of ADAMTS13 --> increase in vWF
FAT RN (fever, anemia, thrombocytopenia, renal failure, neurological symptoms)
Treatment = plasma exchange
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7
Q

Disseminated Intravascular Coagulation (DIC)

A

DDIC = D dimer elevated, dripping (oozing), ill, clots
Increased PT/PTT
Increased D dimer
Decreased fibrinogen

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8
Q

Hemolytic Uremic Syndrome (HUS)

A

HUSS = hamburger, urinary symptoms, shitting, school-aged
E. coli 0157:H7
Elevated serum creatinine

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9
Q

Immune Thrombocytopenic Purpura (ITP)

A 
More common in females (20-40 yo)
Autoantibodies against platelets
Children = after viral infection
Adult = chronic
Diagnostics = isolated thrombocytopenia, + direct Coombs, NO schistocytes
Treatment = steroids, IVIG
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10
Q

Heparin Induced Thrombocytopenia (HIT)

A

Heparin exposure in last 5-10 days
Thrombosis
Thrombocytopenia –> 50% decrease after heparin initiation
Treatment = STOP heparin and START non-heparin anticoagulation (Argatroban)

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11
Q

Low Molecular Weight Heparin

A

Anything that ends in -PARIN

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12
Q

Three Types of Anemia

A

Microcytic (MCV < 80)
Normocytic (MCV 80-100)
Macocytic (MCV > 100)

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13
Q

Etiologies of Microcytic Anemia

A

TICS

  • Thalessemia
  • Iron deficiency
  • Chronic disease
  • Sideroblastic
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14
Q

Etiologies of Normocytic Anemia

A

Chronic disease
Sickle cell
G6PD deficiency
Hereditary spherocytosis

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15
Q

Etiologies of Microcytic Anemia

A

Vitamin B12 deficiency

Folate deficiency

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16
Q

Ferritin

A

Stored iron

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17
Q

Transferrin (TIBC)

A

Binds to and transports iron

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18
Q

HgA

A

Primary global in adults

2 alpha + 2 beta chains

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19
Q

Iron Deficiency Anemia

A

Most common cause of anemia
Etiologies = blood loss and decreased intake/absorption
Diagnostics = decreased iron, decreased ferritin, increased TIBC

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20
Q

Sideroblastic

A 
Accumulation of iron in the mitochondria
Diagnostics = iron increased, ferritin decreased, TIBC decreased
Ringed sideroblasts (SIDE of the O is BLASTed with iron)
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21
Q

Thalessemia

A

Abnormality in global production
Alpha or Beta
Diagnostics = hemoglobin electrophoresis

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22
Q

Alpha Thalessemia

A

1 deletion = asymptomatic
2 deletions = mild anemia
3 deletions = severe anemia (transfusion dependent), HbH
4 deletions = hydros fetalis (incompatible with life), Hb Barts

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23
Q

Beta Thalssemia

A

Minor (1 deletion) or major (2 deletions)

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24
Q

Beta Thalessemia Minor

A

Mild anemia

Usually asymptomatic

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25
Beta Thalessemia Major
``` Severe anemia Extramedullary hematopoiesis Frontal bossing Hair on end appearance on skull x-ray Chipmunk facies Diagnostics = Increased HgA2, HbF ``` Beta fish are the BOSS and their fins have hair on end appearance
26
Anemia of Chronic Disease
Microcytic OR normocytic Etiologies = RA, CKD, IBD, DM, malignancy Diagnostics = iron decreased, ferritin increased, TIBC decreased
27
Sickle Cell
Most common in African Americans Pathophysiology = valine replaces glutamic acid (VAL kicks butt with her big GLUTS) Diagnostics = hemoglobin electrophoresis --> HbS, decreased HgA Treatment = hydroxyurea (for prevention)
28
G6PD Deficiency
Pathophysiology = decreased G6PD = no conversion of NADP to NADPH Symptoms present under stress (G5PD): - Green at the gills (infection) - Fava beans - Primaquine - Dapsone X-linked recessive Diagnostics = peripheral smear --> Heinz bodies, bite cells (STRESS makes me eat BITES of fries with HEINZ ketchup)
29
Hereditary Spherocytosis
Pathophysiology = dysfunctional RBC skeleton proteins --> increased fragility of RBCs and alteration of biconcave to sphere-shaped RBCs Clinical presentation = splenomegaly, jaundice Diagnostics = MCHC elevated (36+), EMA binding > osmotic fragility
30
Vitamin B12 Deficiency
Pernicious anemia = most common cause Clinical presentation = neurological symptoms Diagnostics = MMA increased (you must B12 to watch MMA)
31
Pernicious Anemia
Autoimmune destruction of gastric parietal cells Problem with parietal cells = problem with intrinsic factor (IF) IF responsible for transporting vitamin B12
32
Folate Deficiency
Etiologies = diet (chronic alcoholics, elderly) and pregnancy Clinical presentation = NO neurological symptoms Diagnostics = MMA normal
33
Hemophilia A
Factor VIII deficiency Male PTT prolonged Treatment = DDAVP and factor VIII infusions
34
Hemophilia B
``` Factor IX deficiency Male PTT prolonged Factor IX infusions NO DDAVP* ```
35
von Willebrand Disease
Most common inherited bleeding disorder Decrease in quality/quantity of vWF Clinical presentation = mucocutaneous bleeding, excessive bruising Treatment = DDAVP, vWF concentrates
36
Factor V Leiden
Most common cause of inheritable hypercoaguable state Single point mutation in factor V Clinical presentation = VTE, miscarriages Treatment = anticoagulation
37
Protein C and S Deficiency
Protein S and C --> Stop Clots Deficiency = hypercoaguable state Clinical presentation = VTE, warfarin-induced necrosis Treatment = anticoagulation, protein C concentrate
38
Common Pathway
Factors I, II, V, X
39
Extrinsic Pathway
Factors III, VII | PT (play tennis outside)
40
Intrinsic Pathway
Factors VII, IX, XI, XII | PTT (play table tennis inside)
41
Hodgkin Lymphoma (6 B's)
``` B cell malignancy B symptoms Bound in place (contiguous spread - better prognosis) Binucleated cells (Reed-Sternberg) Bimodal age distribution eBv infection ```
42
Most common Hodgkin Lymphoma
Nodular sclerosing (70%)
43
Hodgkin Lymphoma Treatment
Chemo/radiation (ABVD)
44
Non Hodgkin Lymphoma
``` ABSENCE of Reed-Sternberg Cells Extranodal involvement (poor prognosis) ```
45
Most Common Extranodal Site for Non Hodgkin Lymphoma
GI tract
46
Non Hodgkin Lymphoma Treatment
Chemo/radiation (R-CHOP)
47
Most common type of Non Hodgkin Lymphoma
Diffuse Large B Cell
48
Burkitt's Lymphoma
Starry sky appearance EBV Jaw/facial bone tumor Endemic Africa *Guy holding a control bar while kite surfing at night on the coast of Africa when Jaws appears.*
49
Lymphoma Diagnostics
Excisional biopsy | PET/CT scan
50
Multiple Myeloma - Pathophysiology
Proliferation of a single clone of plasma cell (IgG, IgA)
51
Multiple Myeloma - Risk Factors
> 65 yo African American Men
52
Multiple Myeloma - Clinical Presentation
BREAK - Bone pain - Recurrent infections - Elevated Ca++ - Anemia - Kidney injury CRAB - Ca++ elevated - Renal impairment - Anemia - Bone pain
53
Multiple Myeloma - Diagnostics
Serum protein electrophoresis --> monoclonal spike protein Urine protein electrophoresis --> Bence-Jones proteins Skull x-ray --> "punched out" lytic lesions Bone marrow aspiration --> plasmacytosis (> 10%)
54
Multiple Myeloma - Treatment
Autologous stem cell transplant
55
Polycythemia Vera - Pathophysiology
Overproduction of all 3 myeloid stem cell lines (primarily RBCs) JAK2 mutation
56
Polycythemia Vera - Risk Factors
50-60 yo | Men
57
Polycythemia Vera - Clinical Presentation
*Pruritus* --> especially after hot shower/bath Hepatosplenomegaly Facial flushing
58
Polycythemia Vera - Diagnostics
3 major or 2 minor plus 1 major Major: - Increased RBC mass - Bone marrow biopsy --> hypercellularity - Presence of JAK2 mutation Minor: - Decreased serum EPO - Increased leukocyte alkaline phosphatase - Normal O2 sat - Iron deficiency - Increased granulocytes, WBCs, platelets, B12
59
Polycythemia Vera - Treatment
Phlebotomy Low dose aspirin IF high risk = hydroxyurea, ruxolitinib
60
Myelodysplastic Syndrome (MDS)
Preleukemic disorder Abnormal differentiation of cells of the myeloid cell line > 65 yo Clinical presentation = asymptomatic pancytopenia Diagnostics: - CBC with peripheral smear --> decrease in 1+ myeloid cell line - Bone marrow biopsy --> dysplastic, increased myeloblasts with < 20% pseudo-pelter-Huet cells
61
Leukopenia
Decreased WBCs Agranulocytosis --> may be caused by methimazole Causes = viruses, chemo, steroids, SLE, clozapine CBC = WBC > 3.7, neutropenia, ANC < 1.5 Treatment = discontinue causative agent
62
Hereditary Hemochromatosis
Excess iron deposition in heart, liver, pancreas, endocrine organs C282Y HFE Decreased hepcidin = Increased intestinal iron absorption Clinical presentation = bronze/metallic skin Diagnosis = liver biopsy, increased hemosiderin Treatment = phlebotomy
63
Autoimmune Hemolytic Anemia
Increased destruction of erythrocytes due to presence of anti-erythrocyte antibodies (AEA) Clinical presentation = asymptomatic, fatigue, SOB, pallor, jaundice Treatment = corticosteroids, Ig/splenectomy if refractory
64
Autoimmune Hemolytic Anemia - Diagnostics
``` Decreased Hgb, Hct Increased reticulocytes Increased LDH Decreased haptoglobin Increased indirect bilirubin + direct Coombs ```
65
Lead Poisoning - Pathophysiology
Acquired sideroblastic anemia | < 6 yo
66
Lead Poisoning - Clinical Presentation
Neurologic: - Ataxia - Fatigue - Learning disabilities - Difficulty concentrating - Peripheral neuropathy (wrist/foot drop) GI: - Lead colic --> intermittent abdominal pain, V, loss of appetite, constipation
67
Lead Poisoning - Diagnostics
Serum lead > 10 mcg/dL Peripheral smear --> microcytic hypo chromic anemia, basophilic stippling Bone marrow biopsy --> ringed sideroblasts X-rays --> lead lines
68
Lead Poisoning - Treatment
``` Removal of source IF moderate (45-69 mcg/dL) --> succiner ```
69
Hemolytic Anemias - Diagnostics
``` Increased reticulocyte count Increased LDH Increased indirect bilirubin Decreased haptoglobin Decreased Hct, Hgb ```
70
Pernicious Anemia
``` Pathophysiology = autoantibodies against intrinsic factor Diagnostics = + schilling test, MCV > 100, hyperhsegmented neutrophils, decreased IF, decreased parietal cell antibodies Treatment = B12 IM monthly ```

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