hematolgy 2 Flashcards
When to suspect hereditary thrombophilia?
age < 45 w/o another risk factor
causes?
actor V leiden mutasion
prothrombin mutation
antithrombin deficiency
protein c and s deficiency
actor V leiden mutasion?
MCC
activated resistance to protein C
prothrombin mutation?
2nd MCC
Increase prothrombin
antithrombin deficiency?
Inherited form rare
acquired: cirrhosis, nephrotic syndrome and DIC
Protein C and S deficiency?
decrease inactivation of factor V and VIII
warfarin induced skin necrosis
Triads of hereditary spherocytosis?
Jaundice Splenomegaly Hemolytic anemia Hemoglobinuria Increase MCHC
cancer-related anorexia/cachexia syndrome?
anorexia
wt loss
decrease skeletal muscle mass
management?
progestrone analog(megestrol acetate)-1st line corticosteroid:2nd line B/C side effect
what about cannabinoids?
Effective for HIV related anorexia
age >45 with no identified risk factor for TE develop TE first test?
Age-appropriate cancer screening
Lab finding in antiphospholipid syndrome?
antiphospholipid antibody
increase PTT not respond to plasma mixing
respond to phospholipid mixing that bind adequately to Ab
CLL?
tumor due to mature B cell proliferation
CM?
lymphadenopathy(cervical, supraclavicular and axillary)
Hepatosplenomegaly
mild thrombocytopenia and anemia
Diagnosis?
Sever lymphocytosis
Smug cell
Flow cytometry
LN and BM biopsy not needed
complication?
Infection
autoimmune hemolytic anemia
secondary malignancy
Infection?
due to hypogammaglobulinemia and defective cell signaling
secondary malignancy?
Richter transformation
Richter transformation
also known as Richter’s syndrome (RS),
is a transformation of B cell chronic lymphocytic leukemia (CLL) or hairy cell leukemia into a fast-growing diffuse large B cell lymphoma, a variety of non-Hodgkin lymphoma which is refractory to treatment and carries a bad prognosis.
Prognosis?
Median survival is 10 year Poor prognosis sign multiple chain LDP Hepatosplenomegaly anemia and thrombocytopnia
Cause of microcytic anemia?
IDA
Thalassemia
Heme structure defect(HC,S,CS) disease
Lead poisoning
IDA and Lead poisoning similar laboratory findings?
Low iron and TIBC will be low
If iron and TIBC will be normal?
Suscepect Thalassemia and Heme structure defect
what to do then?
electrophoresis
Epidemiology of ITP?
commonly acquired
auto Ab formation
recent viral infection and comorbidity(HCV
Manifestation?
Usually asymptomatic
Mucocutaneous bleeding
ecchymosis,petichia and purpura
severe hemorrhage is rare
Diagnosis?
diagnosis of exclusion
What to exclude?
Drug liver disease hypersplenism Microangiopathic disorder BM disorder
the drug should be given with phenytoin?
Folic acid
Ca
Vit D
management of ITP?
depends on the platelet count
> 30,000
observation
< 30,000
corticosteroid
massive bleeding?
Iv Ig and platelet transfusion
transfusion and hypocalcemia?
blood contain citrate which can chelate Ca and cause hypocalcemia massive transfusion(> 1 volume) per 24 hour
Traumatic macrovascular hemolysis sign?
anemia jaundice hepatosplenomegaly(work hyperthrophy) mild thrombocytopenia schistocytes on pheripherial morphology
why G6PD should be considered in times of crisis even with a negative G6PD enzyme assay?
It has low sensitivity in time of acute crisis
what do we use to diagnose at this time?
sign of hemolytic anemia
inciting event
bite cell and Henze body
most common origin of PE thrombi?
pelvic/femoral vein
giving the only folate in case of cobalamin deficiency?
Improve anemia but precipitate the neurologic symptoms.
Febrile non-hemolytic transfusion rxn?
fever, chills, and rigor with 1-6 Hr after transfusion
Transient
respond to antipyretic
prevention?
Lukoreduction for blood storage
Pathogenesis
cytokine released by leukocytes in donor blood/
other benefits of leukoreduction?
Prevent HLA alloimmunization
CMV transmission
AML?
MC adult leukemia
Median age 65
CM?
fatigue(B-Symptom rare) symptom of cytopenia anemia-fatigue and weakness bleeding and bruising from thrombocytopenia infection(granulocytopnia) hepatosplenomegaly and LDP rare DIC(If APL)
Laboratory?
Cytopenias? maybe leukocytosis
Elevated LDH
Pheripherial smear: promyelocyte
elevated PT and PTT
Diagnosis?
BM biopsy: Hypercelular with myeloid blast
why MM patients are at risk of infection?
defective Ab production
What will be RDW and RSC count in thalassemia?
Normal
