Hematology (4%) Flashcards
Acute Lymphocytic Leukemia (ALL)
Most common childhood cancer (ALL kids have cancer)
ALL = Anemia, lumps, limp
Bone marrow biopsy = > 20% lymphoblasts
CNS prophylaxis with intrathecal methotrexate
Acute Myeloid Leukemia (AML)
Middle aged
Acute Promyelocytic Leukemia (APL) = most common subtype
Bone marrow biopsy = > 20% myeloblasts
Acute Promyelocytic Leukemia (APL)
Auer rods (A-rod plays in the big APL) Treatment = chemo PLUS Vitamin A
Chronic Lymphocytic Leukemia (CLL)
Older adults (70 yr) Smudge cells (CLL --> Crushed Little Lymphocytes) IF asymptomatic --> observe
Chronic Myeloid Leukemia (CML)
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.
Thrombotic Thrombocytopenic Purpura (TTP)
Severe deficiency of ADAMTS13 --> increase in vWF FAT RN (fever, anemia, thrombocytopenia, renal failure, neurological symptoms) Treatment = plasma exchange
Disseminated Intravascular Coagulation (DIC)
DDIC = D dimer elevated, dripping (oozing), ill, clots
Increased PT/PTT
Increased D dimer
Decreased fibrinogen
Hemolytic Uremic Syndrome (HUS)
HUSS = hamburger, urinary symptoms, shitting, school-aged
E. coli 0157:H7
Elevated serum creatinine
Immune Thrombocytopenic Purpura (ITP)
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
Heparin Induced Thrombocytopenia (HIT)
Heparin exposure in last 5-10 days
Thrombosis
Thrombocytopenia –> 50% decrease after heparin initiation
Treatment = STOP heparin and START non-heparin anticoagulation (Argatroban)
Low Molecular Weight Heparin
Anything that ends in -PARIN
Three Types of Anemia
Microcytic (MCV < 80)
Normocytic (MCV 80-100)
Macocytic (MCV > 100)
Etiologies of Microcytic Anemia
TICS
- Thalessemia
- Iron deficiency
- Chronic disease
- Sideroblastic
Etiologies of Normocytic Anemia
Chronic disease
Sickle cell
G6PD deficiency
Hereditary spherocytosis
Etiologies of Microcytic Anemia
Vitamin B12 deficiency
Folate deficiency
Ferritin
Stored iron
Transferrin (TIBC)
Binds to and transports iron
HgA
Primary global in adults
2 alpha + 2 beta chains
Iron Deficiency Anemia
Most common cause of anemia
Etiologies = blood loss and decreased intake/absorption
Diagnostics = decreased iron, decreased ferritin, increased TIBC
Sideroblastic
Accumulation of iron in the mitochondria Diagnostics = iron increased, ferritin decreased, TIBC decreased Ringed sideroblasts (SIDE of the O is BLASTed with iron)
Thalessemia
Abnormality in global production
Alpha or Beta
Diagnostics = hemoglobin electrophoresis
Alpha Thalessemia
1 deletion = asymptomatic
2 deletions = mild anemia
3 deletions = severe anemia (transfusion dependent), HbH
4 deletions = hydros fetalis (incompatible with life), Hb Barts
Beta Thalssemia
Minor (1 deletion) or major (2 deletions)
Beta Thalessemia Minor
Mild anemia
Usually asymptomatic
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
Anemia of Chronic Disease
Microcytic OR normocytic
Etiologies = RA, CKD, IBD, DM, malignancy
Diagnostics = iron decreased, ferritin increased, TIBC decreased
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)
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)
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
Vitamin B12 Deficiency
Pernicious anemia = most common cause
Clinical presentation = neurological symptoms
Diagnostics = MMA increased (you must B12 to watch MMA)
Pernicious Anemia
Autoimmune destruction of gastric parietal cells
Problem with parietal cells = problem with intrinsic factor (IF)
IF responsible for transporting vitamin B12
Folate Deficiency
Etiologies = diet (chronic alcoholics, elderly) and pregnancy
Clinical presentation = NO neurological symptoms
Diagnostics = MMA normal
Hemophilia A
Factor VIII deficiency
Male
PTT prolonged
Treatment = DDAVP and factor VIII infusions
Hemophilia B
Factor IX deficiency Male PTT prolonged Factor IX infusions NO DDAVP*
von Willebrand Disease
Most common inherited bleeding disorder
Decrease in quality/quantity of vWF
Clinical presentation = mucocutaneous bleeding, excessive bruising
Treatment = DDAVP, vWF concentrates
Factor V Leiden
Most common cause of inheritable hypercoaguable state
Single point mutation in factor V
Clinical presentation = VTE, miscarriages
Treatment = anticoagulation
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
Common Pathway
Factors I, II, V, X
Extrinsic Pathway
Factors III, VII
PT (play tennis outside)
Intrinsic Pathway
Factors VII, IX, XI, XII
PTT (play table tennis inside)
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
Most common Hodgkin Lymphoma
Nodular sclerosing (70%)
Hodgkin Lymphoma Treatment
Chemo/radiation (ABVD)
Non Hodgkin Lymphoma
ABSENCE of Reed-Sternberg Cells Extranodal involvement (poor prognosis)
Most Common Extranodal Site for Non Hodgkin Lymphoma
GI tract
Non Hodgkin Lymphoma Treatment
Chemo/radiation (R-CHOP)
Most common type of Non Hodgkin Lymphoma
Diffuse Large B Cell
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.
Lymphoma Diagnostics
Excisional biopsy
PET/CT scan
Multiple Myeloma - Pathophysiology
Proliferation of a single clone of plasma cell (IgG, IgA)
Multiple Myeloma - Risk Factors
> 65 yo
African American
Men
Multiple Myeloma - Clinical Presentation
BREAK
- Bone pain
- Recurrent infections
- Elevated Ca++
- Anemia
- Kidney injury
CRAB
- Ca++ elevated
- Renal impairment
- Anemia
- Bone pain
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%)
Multiple Myeloma - Treatment
Autologous stem cell transplant
Polycythemia Vera - Pathophysiology
Overproduction of all 3 myeloid stem cell lines (primarily RBCs)
JAK2 mutation
Polycythemia Vera - Risk Factors
50-60 yo
Men
Polycythemia Vera - Clinical Presentation
Pruritus –> especially after hot shower/bath
Hepatosplenomegaly
Facial flushing
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
Polycythemia Vera - Treatment
Phlebotomy
Low dose aspirin
IF high risk = hydroxyurea, ruxolitinib
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
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
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
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
Autoimmune Hemolytic Anemia - Diagnostics
Decreased Hgb, Hct Increased reticulocytes Increased LDH Decreased haptoglobin Increased indirect bilirubin \+ direct Coombs
Lead Poisoning - Pathophysiology
Acquired sideroblastic anemia
< 6 yo
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
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
Lead Poisoning - Treatment
Removal of source IF moderate (45-69 mcg/dL) --> succiner
Hemolytic Anemias - Diagnostics
Increased reticulocyte count Increased LDH Increased indirect bilirubin Decreased haptoglobin Decreased Hct, Hgb
Pernicious Anemia
Pathophysiology = autoantibodies against intrinsic factor Diagnostics = + schilling test, MCV > 100, hyperhsegmented neutrophils, decreased IF, decreased parietal cell antibodies Treatment = B12 IM monthly
