Card Set Information
M/C cause of anemia in the world
m/c cause of IDA (in US)
What is the definition of severe IDA?
Hct < 25%
PBS of IDA
Later hypochromic microcytic red cells, anisocytosis, and poikilocytosis
Hgb H dz
only one alpha chain present
B thall major
When is Cooley's anemia dx?
problems begin at 4-6 months, when the switch from fetal Hgb to adult Hgb
How to differentiate b/w thalassemia and IDA
Serum iron and ferritin levels are nl
How do you dx thalassemia?
How do you tx thalassemia?
folic acid supplements, avoid iron suppleemnts and oxidative meds
How do you avoid hemosiderosis in thalassemia tx?
What type of anemia is sideroblastic anemia?
How do you dx sideroblastic anemia?
Prussian Blue staining
What are causes of sideroblastic anemia?
myelodysplasia, chronic alcoholism, and lead poisoning
m/c cause of normochronmic normocytic anemias
Te cell meidate autoimmune suppression of hematopoesis
S/Sx of aplastic anemia
weakness, fatigue, vulnerability to infection, pallor, purpura, and petechiae
what is the hallmark of aplastic anemia?
What type of anemia is aplastic anemia?
m/c cause of folic acid deficiency
poor dietary intake
What meds are folic acid antagonists?
Phenytoin, Bactrim, sulfasalazine
What is the daily requirement of folic acid?
What disease states increase folic acid requirements?
Pregnancy, hemolytic anemias, and exfoliative skin dz
What is pathognominc for folic acid deficiency?
Macro-ovalocytes and hypersegmented PMNs
What does a PBS of folic acid deficiency look like?
macroovalocytes, hypersegmented PMN, Howell-Jolly bodies
What are Howell-Joly bodies?
nuclear DNA remnants
How do you manage folic acid?
what is the m/c cause of B12 anemia
Where does absorption of B12 occur?
terminal ileum, storage in the liver
S/Sx of B12 deficiency
glossitis, pale icterus, vague GI sx
: stocking-glove paresthesias, loss of position, fine touch and vibratory sensation, clumsiness, dementia, and ataxia
PBS of B12 deficiency
anisocytosis, poikilocytosis, macro-ovalocytosis, and hypersegmented neutrophils
Lab findings of B12 deficiency
Reduced reticulocyte count, elevated LDH and indirect bilirubin, low B12
Tx of b12
lifelong Im injections for pernicious anemia, daily oral cobalmin in high doses
When are B12 neurological sx reversible?
Treated within 6 months
Name intrinsic hemolytic anemias
hereditary spherocytosis and elliptocytosis, G6PD, methemoglobinemia, SCA
What are extrinsic causes of hemolytic anemia?
autoimmune and lymphoproliferative diseases, drug toxicity, TTP, HUS, DIC, valvular hemolysis, metastatic adenocarcinoma, vasculitides, infections, hypersplenism, burns
Presentation of hemolytic anemias
jaundice, gallstones, pallor, and sx
Infection with what pathogen can cause transient aplastic crisis?
What is the hallmark of hemolytic anemia?
elevated reticulocyte count in presence of falling or stable HCT
PBS of hemolytic anemia
immature red cells, nucleated red cells, nucleated red cells, or morphologic changes
What are lab findings in hemolytic anemia?
transient hemoglobinemia, elevated unconjugated bili, serum LDH high (microangiopathic hemolysis)
Genetics of SCA
What is pathophys of SCA?
RBCs containing primarily HGB S sickle under deoxygenated conditions
What increases sickling in SCA?
red cell dehydration, acidosis, hypoxemia, high altitudes
People with SCA are at risk for what?
cholelithiasis, splenomegaly, poorly healing ulcers, infection with encapsulated organisms, strokes, priapism, retinopathies, osteomyelities, avascular necrosis
How do you dx SCA?
HGB electrophoresis, PBS
PBS of SCA
sickled cells and target cells, nucleated RBCs, HJ bodes
Lab findings of SCA
elevated tic count
Elevated wbC count
thrombocytosis may be present
indirect bili may be elevated
How do you tx SCA?
Analgesics, fluids, oxygen
transfusions for vaso-occlusive crises
Pneumococcal vaccine q10 years, folate supplementation
Genetics of G6PD
x-linked recessive disorder commonly seen in American black males and some mediterreanean populations
List oxidative drugs
ASA, dapsone, primaquine, quinidine, sulfonamides, nitrofurantoin)
Lab findings of G6PD
tic count and serum indirect bili increase during hemolytic episodes
PBS reveal bite cells and Heinz bodies
G6PD levels low
PBS of G6PD
bite cells and Heinz bodies
Tx of G6PD
self-limited as RBCs are replaced
avoid oxidative drugs
What is PCV?
slowly progressive bone marrow disorder characterized by increased numbers of RBCs and increased total blood volume;
unregulated expansion of RBC causes hyperviscosity, which leads to decreased cerebral blood flow
What is dx of PCV?
presence of JAK2 mutation
m/c cause of mortality/morbidity in PCV
epidemiology of PCV
M/F, median age of presentation 60%
Avg survival time of someone with PCV
PCV can convert to what?
myelofibrosis or CML, rarely to AML
What are dx criteria for PCV?
splenomegaly, nl arterial O2 sat, elevated RBC mass
Sx of PCV
sx of increased blood viscosity (HA, dizziness, fullness in head and face, weakness, fatigue, tinnitus, blurred vision); burning, pain, redness of extremities, stroke
Pruritus after bathing
What are common comorbidities with PCV?
epistaxis (may be presenting sx)
plethora, systolic HTN, engorged retinal veins, splenomegaly
Lab findings in PCV
HCT > 54% males, 51% females
PBS shows neutrophilic leukocytosis, increased basophils and eosinophils, increased numbers of large, bizarre plts
nl RBC morphology
hypercellular bone marrow in all cell lines
neutrophilic leukocytosis, increased basophils and eosinophils, increased numbers of large, bizarre plts
How do you tx PCV?
phlebotomy is tx of choice
myelosuppressive therapy with hydroxyurea may be indicated; anagrelide may be added or substituted
low dose ASA reduces risk of thrombosis without increasing the risk of bleeding
what is leukemia?
diseases characterized by unrestrained growth of leukocytes and leukocyte precursors in the tissues
M/c childhood leukemia
age range of ALL
Age range of AML
avg age of onset 60 yo
Clinical features of acute leukemias
gingival bleeding, epistaxis, menorrhagia; DIC is less common
fatigue, abrupt onset of fever, lethargy, HA, and bone and or jt pain (esp in sternum, tibia, and femur)
what is the hallmark of acute leukemia?
pancytopenia with circulating blasts; blasts make up at least 20% of nucleated cells in bone marrow
What are lab findings in acute leukemia?
pancytopenia with circulating blasts
Auer rods in AML
Leukmia with mediastinal mass
Presence of Philadephia chromosome is unfavorable
What is CLL?
clonal malignancy of B lypmhocytes
What is the m/c leukemia?
What is the m/c form of CLL?
B cell form
What is the rai system?
prognostic staging for CLL
Epidemiology of CML
young to middle aged adults (mediat age is 55 years)
Three phases of CML
chronic, accelerated, and acute (aka blast crisis, >30% blasts)
Sx of CML
fatigue, anorexia, weight loss, low-grade fever, excessive sweating
abdominal fullness caused by splenomegaly
runs a mild course until blast phase
clinical features of CLL
peripheral lymphocytosis and lymphotic invasion of bone marrow, liver, spleen, and LNs
recurrent infections, HSM
a type of CLL, where an isolated node transforms into aggressive, large cell lymphoma
What is the hallmark of CLL?
isolated lymphocytosis (leukocytosis > 20,000 cells)
What is the hallmark of CML?
leukocytosis, with meadian WBC count of 150,000
gene used to idenitfy chronic leukemia
anemia and thrombocytosis
mature small lymphocytes, smudge cells
Tx of CML
Gleevec, dastinib or nilonib in cases of Gleevec intolerance
allogenic bone marrow transplant may bt eh initial tx and is the only therapy proven to be curative
Tx of Acute leukemias
chemotherapy, consolidation therapy
bone marrow transplant if poor reponse to tx
Tx of CLL
pallaitive once dz is advanced
group of cancers characterized by enlargement of lymphoid tissue, spleen, and liver and the presence of reed-sternberg cells
Reed Sternberg cells
Virus associated with Hodgkin's Disease
Age range Hodgkin's disease
15-45 yo, peaking in 20s, and again after 50; rare under 5 yo
Clinimcal features of Hodgkin's disease
painless cervical, supraclavicular, and mediastinal lymphadenopathy; pain in affected node after ingestion of alcohol
Stage A Hodgkins
lack of constituational sx
What indicated a poorer prognosis in HD?
presence of constitutional sx
Staging system for Hodgkins and NHL
Tx of HD
radiation therapy nitial tx of choice for pts with low risk stage IA and IIA dz
most other pts receive adriamycin, bleomycin, vinblastine, and dacarbazine chemo (ABVD)
group of malignancies that arise from lymphocytes, usually from B lymphocites
epidemiology of NHL
Peak incidence between 20 and 40 yo, lymphomas divided into clinically indolent and addgressive groups
Clinical features of NHL
diffuse or isolated, painless, persistant lymphadenopathy is the m/c presentation, extralymphatic presentation, some constitutaionl sx
m/c presentation of NHL
diffuse or isolated, painless, persistant lymphadenopathy
Extralymphatic presentations of NHL
GI tract, skin, bone, and bone marrow
M/c presentation of burkitt's lymphoma
What is used as a prognostic marker for NHL?
Tx of NHL
some spontaneous remission
single involved node
low grade lymphoma
: rituximab with or without chemo
: Low grade lymphoma=allogenic transplant
Intermediate or high grade lymphomas
: chemo and autlogous stem cel transplant
malignancy of plasma cells, possibly caused by HPV
replacement of bone marrow leads to failure, bone destruction leads to pain, OP, lytic lesions, hypercalcemia, and pathologic features
Why are MM pts prone to recurrent infections?
Neutropenia and failure of Ab production in reponse to antigen challenge
median age of dx of MM
M/c presenting complaints of MM
anemia, bone pain (particularly in low back or ribs, and infection)
also renal failure, spinal cord comperssion, and hyperviscosity syndrome
Monoclonal spike on protein electrophoresis
lytic lesions on xray with generalized Op
Tx of MM
lenalidomide, dexamethasone, and doxorubicin; with bisphosphonates as adjuncts
What does thrombin clotting time measure?
rate of conversion between fibrinogen and fibrin in the presence of thrombin
m/c cause of abnl bleeding
Causes of TCP
impaired production, increased destruction, splenic sequestration, or dilution
self limited, auto-immune disorder found commonly in children of both sexes and is associated with a preceeding viral URI
Associated with URI
Sx of acute ITP
abrupt appearance of petechaie, purpura, and hemorrhagic bullae
Chronic ITP presentation
petechiae on skin and mucous membranes
What drug m/c causes ITP type reaction?
what other drugs can cause ITP?
sufulonamides, quinine, thiazides, cimetidine, and gold
Lab findings of acute ITP
decreasd plalets (b/w 10-20k), eosinophilia, and mild lymphocytosis
mild anemia, nl coag
lab findings of chornic ITP
plts b/w 25-75k
mild anemia, nl coag
Tx of acute ITP
usually resolves spontaneously, some pts require corticosteroids or splenectomy
chronic ITP tx
high dose prednisone
If tx fails
: IVIG, danazol, immunosuppresive therapy, stem cell transplant
Three types of plt consumption syndromes
TTP, HUS, and DIC
Epidemiology of TTP
20-50 yo, m/c in women than men, common in HIV
What can precipitate TTP?
Estrogen use, pregnancy, drugs such as quinine and ticlopidine
What is TTP?
Abs against ADAMTS13 enzyme, which breaks down large multimers of VWF. therefore an increase in VWF leads to an increase in clotting at sites of vascular injury
What is DIC?
generalized hemorrhages in pts with severe underlying systemic illness such as sepsis, tissue injury, obstetric complications, cancer, and severe transfusion reactions
What is the TTP pentad? What are other sx?
purpura, microangiopathic hemolytic anemia, fever, abnl neurologic signs, renal failure
petechiae, apllor, abdominal pain, possibly pancreatitis
What is HUS?
Child version of TTP without neurologic findings, but with more kidney problems
Who does HUS affect?
<10 yo, particularly after infection with E coli, shigella, salmonella, and various viruses
What are s/sx of DIC?
skin and mucous membrane bleeding (particularly at puncture/wound sites), shock, thrombosis
What are lab findings of TTP?
anemia, red cell fragmentation (Schistocytes), nl leukocytes, polychromaphilia, reticulocytosis, and TCP (less severe in HUS than in TTP), negative Coombs
High indirect bili
What are lab findings of HUS?
anemia, red cell fragmentation (Schistocytes), nl leukocytes,
polychromaphilia, reticulocytosis, and TCP (less severe in HUS than in
TTP), negative Coombs
High indirect bili
What are lab findings in DIC?
Evidence of coagulopathy (hypofibrinogenmia, elevated fibrin degradation products)
Microangiopathic hemolytic anemia with schistocytes
Tx of TTP
large volume plasmapheresis
prednisone and antiplatelt agents
Tx of HUS
fluids and management of electrolyte imbalance
in adults, plasmapheresis
tx underlying cause
component blood transfusions are important; role of heparin is controversial
m/c cause of acquired plt dysfunction
Genetics of VWD
m/c congenital coagulopathy
Clinical features of vWD
bleeding in nasal, sinus, vaginal, and GI mucous membranes
rare bleeding into joint spaces
lab findings in vWD
nl pt/ptt, prolonged bleeding time
How do you tx VWD?
vasopressin for type I
factor VIII concentrates are preferred if factor replacement is necessary
Factor VIII deficiency
What is hemophilia a?
a hereditary disease caused by excessively prolonged coagulation time
genetics of hemophilia A
x linked recessive
most severe bleeding disorder
Clinical features of Hemophilia A
Spontaneous hemorrhagic episodes with hemearthroses, epistaxis, intracranial bleeding, hematemesies, melena, microscpoic hematuria, bleeding into soft tissue and gingiva
excessive bleeding following trauma/surgery
Lab findings in Hem A
PT, bleeding time, fibrinogen level, plts nl
reduced factor 8, vwf nl
How do you tx Hem A?
infusion of heat treater or recombinant factor 8
desmopressin may elevated levels in mild to mod dz
avoid ASA, use celecoxib or opiods for pain
Factor 9 deficiency
genetics of Hem B
X linked recessive disorder
Favtor XI deficiency
found in Ashkenazi Jews
Most common acquired coagulopathies
What are causes of Vit K deficiencies?
use of some drugs
Labs in Vit K dependent factor deficiencies
PT prolonged, PTT may be prolonged
Fibrinogen, thrombin time, plt count are nl
liver enzymes may be elevated
Tx of Vit K dependent factor deficiencies
Tx underlying cause
Vit K (PO or IV)
tx hemrorhage with FFP
Vit K dependent factors
2, 7, 9, 10
Acquired hypercoaguable states associated with malignancy