Hematology Flashcards
MCV>100
Macrocytic
MCV
Microcystic
Causes of Low MCV
3 have low reticulocyte count
Iron Def
Thalassemia
Sideroblastic anemia
Anemia of Chronic disease
Causes of Macrocytic Anemia
B12 &Folate Alcholism Sideroblastic Anemia Liver disease/hypothyroidism Meds &Antimetabolite meds MDS
Normocytic Anemia
Acute blood loss and hemolysis
will increase the reticulocyte count.
Anemia Tx?
Packed RBC if severe
Symptomatic Transfuse
Symptomatic Anemia
SOB
Light headed, confused, syncope
Hypotension and tachycardia
Chest pain
Packed RBC raises Hct
by 3 points/unit
FFP replaces
Clotting factors
Microcytic Anemia- Iron Def
GI bleeds/Menstruation Low ferritin Increased TIBC Low serum iron RDW is increased
Microcytic Anemia- Chronic Disease
Cancer/infection/rheumatoid arthritis
High Ferritin- iron trapped
Low TIBC
Low serum iron- all that is floating around is bound
Microcytic Anemia- Sideroblastic
Alcoholic
High serum Iron
Prussian blue staining for ringed sideroblasts
Microcytic Anemia-Thalassemia
typically asymptomatic Target cells Normal iron studies Best test: Hb electrophoresis Normal RDW High reticulocyte
Megaloblastic=Hypersegmented
Seen in ??
Folate and B12 def
Vitamin B12 Def caused by
Pernicious anemia
Pancreatic Insufficiency
Crohn’s
Diphyllobothrium latum
Folate Def. causes
Diet
Psoriasis
Drugs: Phenytoin, sulfa
Alcohol
Macrocytosis and neuro problems
B12 def
Neuro probs- particularly peripherlal neuropathy. Posterior column damage most common
look for ataxia
Labs: Increase methylmalonic acid levels
Pernicious Anemia confirmed with
antiintrinsic factor and anti-parietal cell antibodies
Sickle cell mechanism
Glutamic at 6 position is replaced with valine
Point mutation
Vaso-occlusive crisis caused by
hypoxia
dehydration
infection
cold temperatures
African america
severe chest pain, butt/thigh pain
Fever (?)
Think sickle cell
Tx: with empiric antibiotics if there is a fever.
Sickle Cell Manifestations
Bilirubin gallstones Increased infections--why? Osteomyelitis--common bug? Retinopathy Stroke Skin ulcers Avascular necrosis of femoral head Enlarged hear with hyperdynamic features and systolic murmur
Sickle Cell
- ) Best initial test?
- ) Most accurate test?
- ) Peripheral smear-Howell Jolly Bodies
2. ) Hb electrophoresis
Tx. Sickle Cell
Oxygen/hydration/analgesia Antibiotics if fever Folic acid for all Pneumococall vaccine Hydroxyurea.
Sickle Cell Trait
inability to concentrate urine
No treatment
Hereditary Spherocytosis
Cytoskeleton of RBC mutation
Low MCV
Increased MCHC
best test osmotic Fragility
Young child/newborn with hemolysis Intermittent jaundice Splenomegaly FHx: anemia/hemolysis Bilirubin gallstones
Hereditary Spherocytosis
Tx: folic acid & splenectomy–will stop hemolysis
Autoimmune Hemolysis
aka?
diagnostic test?
Tx:
Warm IgG Coombs test Glucocorticoid therapy Splenectomy IVIG Rituximab
Cold Agglutinin Disease
IgM antibodies against RBC
Direct coombs test
Tx: stay warm, administer rituximab, cyclophosphamide, cyclosporine, plasmapheresis
G6PD
x-linked recessive
inability to generate glutathione reductase
Look for AA/Mediterranea males with sudden anemia/jaundice
Normal spleen
Heinz bodies + bite cells
G6PD
Tx: avoid oxidant stress
HUS/TTP
E. Coli for HUS
Neuro +Fever with TTP
Both have schistocytes, Thromobocytopenia, renal insufficiency
Tx HUS/TTP
Plasmapheresis or plasma exchange
Paroxysmal Noturnal Hemoglobinuria
MCC of death? Thrombosis
def in CD55 and CD59 (best test measures it) causes increased sensitivity to acidosis/hypoxia which occurs at night time. Tx: Prednisone, bone marrow transplant, eculizumab, folic acid and transfusions as needed.
Aplastic Anemia
invasion of bone marrow causing decreased production or hypoplasia
acts as an autoimmune disorder. T-cells attack patients own marrow.
Tx: for Aplastic Anemia
Treat underlying cause
Blood transfusion, antibiotics for infection and platelets for bleeding
BMT if young
Anti-thymocyte globulin and cyclosporine in older.
Polycythemia Vera
JAK2 mutation Hct>60% hypoxia excluded Vit B12 levels are elevated. Iron levels dec. Platelets and WBC count increase Erythropoietin decrease
Essential Throbocytosis
Platelet count>1 million
Tx if older than 60 with plt count>1.5 million
Tx hydroxyurea, aspirin
Myelofibrosis
Fibrosis of the bone marrow
look for teardrop shaped cells
Tx: thalidomide and lenalidomide
Acute Leukemia
ALL M3->DIC
Best test? blood smear
Accurate test? Flow cytometry
Myeloperoxidase–> characteristic for AML
Tx: ALL and AML
Chemo to remove blasts
Remission relapse +poor prognosis= BMT
Remission relapse + good prognosis= chemo
best indicator for acute leukemia
Cytogenetics
good genetics: chemo
bad genetics: BMT
Tx with M3
Add ATRA
Tx with ALL
Intrathecal chemotherapy prevents relapse of all in CNS
CML what to look for
high WBC: all neutrophils
vague symptoms
Splenectomy
Pruritius
Tests with CML
first test: LAP. Will be low in CML
accurate: BCR-ABL the philly chromosome test
CML Tx:
Imatinidb, dasatinib, niotinib best 1st therapy
BMT cures it
Acute Leukostasis reaction
elevated WBC emergency tx with leukapheresis
Myelodysplastic syndrome
Pancytopenia despite hypercellular bone marrow.
MDS labs
anemia with increased MCV, nucleated red cells, small #of blasts
Ringed sideroblasts
Pelger-Huet cells–bilobed nucleus
MDS Tx:
Transfuse
Erythropoietin
Lenalidomide
CLL
WBC elevated with all lymphocytes Lymphadenopathy Spleen/liver enlargement Infection Tx: stage 2+ give fludarabine (+rituximab) PCP prophylaxis refractory: cyclophosphamide mild: chlorambucil severe infection: IVIG
Hairy Cell Leukemia
TRAP positive
Cladribine
Painless Lymphadenopathy
Best test
Excisional biopsy
Nonhodgkin tx
stage 1+2: Local radiation
Adv. stage: chemotherapy (CHOP and rituximab)
Burkitt and immunoblastic bad.
Hodgkin
Reedsternberg cells Lymphocyte predominant best prog. around cervical area Tx: radiation ABVD
Multiple Myeloma
bone pain lytic lesions
don’t light up on bone scan
Plasma cell proliferation IgA and IgG proliferation M spike Bence jones protein Rouleaux
Multiple Myeloma Best test
Bone marrow biopsy >10% plasma cells
Multiple Myeloma Tx
Steriods with lenalidomide, bortezomib, melphalan
MGUS
small number of plasma cells
must get bone marrow biopsy to rule out MM
Waldentrom Macroglobinemia
Overproduction of IgM
Viscosity is a problem
Tx: plasmapheresis best initial therapy
Long term treatment is chlorambucil/fludrabine/prednisone
Platelet Bleeding
Superficial
Factor Bleeding
Deep
Brain bleeds
platelets or factor
Normal HCT, WBC, spleen
ITP
Diagnosis of exclusion
Tx: Prednisone if count
Neisseria
hemophilus
pneumococcus
Vaccinate before splenectomy
Most common inherited bleeding disorder
Von Willebrand Disease autosomal dominant platelet type bleeding with a Normal platelet count worse after aspirin PTT can go up
VWD-tests?
Tx: DDAVP
Bleeding time- increased duration
VWF level will be discreased
Ristocetin cofactor assay
PT normal
Delayed joint of muscle bleeding
Most accurate test: Factor 8 or 9 assay
first study: Mixing study: correct to normal
Hemophila Tx
DDAVP
Factor 11 def.
Tx: FFP if bleeding
With trauma or surgery there is increased bleeding.
Normal PT and prolonged aPTT
Mixing will correct it to normal
DIC RF
sepsis burns snake bites cancer tissue factor release amniotic fluid emboli consumptim coagulopathy
DIC
Tx: FFP
elevated PT and aPTT
elevated D-dimer and fibrin split products
decreased fibrinogen level
low platelet count
2,7,9,10
Vit K factors when def. increase PT and aPTT
Factor V leiden mutation
MCC hypercoaguable state/thrombophilia
HIT
clotting disorder
common with unfractionated heparin
drop in platelet count by 30%
confirmed with ELISA platelet factor 4 or serotonin release assay
Tx: stop heparin switch to Direct thrombin inhibitors (agatroban, lepiruidin, bivalirudin) and then warfarin
Lupus anticoagulant
anti-cardiolipin antibodies
Antiphospholipid syndromes
clotting and increased aPTT
Tx: heparin/warfarin
APL gets lifelong anticoagulation
anti-cardiolipin antibodies
recurrent spontaneous abortions
Antiphospholipid Syndrome with Inc. aPTT
Order what test?
mixing study still elevated
lupus anticoagulant test
russell viper venom test
Tumor Lysis Syndrome
Cell contents leak out
Hyperkalema, hyperphosphotemia, hypocalcemia, hyperuricemia