Hematology Flashcards
how do you dx B12 deficiency and pernicious anemia?
increased MCV >115, hypersegmented neutrophils, increased serum homocysteine, increased methylmalonic acid, decr. B12 levels
if pernicious anemia - + IF antibody, parietal cell Ab, incr. gastrin levels, + Schilling test
what are the macrocytic anemias?
B12 def., folate (vit. B9) def
what is pernicious anemia?
autoimmune destruction/loss of gastric parental cells
what’s the tx for B12 def/pernicious anemia?
IM B12; watch for signs of hypokalemia w/ tx
what’s the s/s of folate (vit. B9) def.?
similar to B12, but NO NEURO SX’s
glossitis***
how do you dx Folate (vit. B9) def?
incr. MCV >115, hyperhsegmented neutrophils, decr. folate, normal B12, incr. serum homocysteine
what’s the tx for folate (vit. B9) def?
folic acid 1mg PO daily
-if neuro sx’s worsen, then it’s B12 def.
what type of anemia is iron def. anemia?
microcytic hypochromic anemia
low MCV, low MCH
what’s the MC cause of iron def. anemia?
blood loss
what’s the dx for iron def. anemia?
decreased ferritin***, increased TIBC, decr. serum iron
increased RDW, decreased transferrin saturation
what’s the tx for iron def. anemia?
iron replacement best on empty stomach (can add vitamin C to increase Fe absorption)
who is alpha thalassemia MC seen in?
SE Asian
what is seen on peripheral smear for alpha thalassemia?
target cells; Heinz bodies in hemoglobin H disease
what’s the tx for mild alpha-thalassemia (alpha-trait)?
no tx
what’s the tx for severe alpha-thalassemia?
- blood transfusions
2. iron chelating agents
who is beta thalassemia MC seen in?
MC in mediterranean
what’s the s/s of beta-thalassemia major?
frontal bossing, sx at 6 months
what’s the peripheral smear show for beta-thalassemia?
target cells; no HgbA
what type of anemia is anemia of chronic disease?
normocytic normochromic anemia
what’s the cause of anemia of chronic disease?
chronic inflammatory conditions
what’s the dx for anemia of chronic disease?
normal or incr. ferritin* + decr. TIBC* (vs Fe def is incr. TIBC), decr. serum Fe
what type of anemia is B12 def/pernicious anemia and folate (vit. B9) def.?
macrocytic normochromic anemia
how do you treat anemia of chronic disease?
treat the underlying disease
what’s the MC cause of G6PD deficiency sx’s?
infections
what’s the MC medications causing G6PD deficiency sx’s?
sulfa drugs (ex. Bactrim), antimalarials
what is G6PD deficiency?
episodic hemolytic anemia
what is seen on peripheral smear for G6PD deficiency?
normocytic hemolytic anemia with schistocytes (“bite” cells)
-+/- Heinz bodies***
what’s the tx for G6PD deficiency?
avoid offending food & drugs
if severe anemia -> Fe/Folic acid supplementation
what’s hemophilia A?
MC type of hemophilia
x-linked recessive trait
lack of factor 8*** -> AFFECTS THE INTRINSIC PATHWAY
what’s the s/s of hemophilia A and B?
Hemarthrosis -> arthropathy
excessive hemorrhage in response to trauma & surgery/incisional bleeding
Deep tissue bleeding in hemophilia B
how do you dx hemophilia A?
low factor 8, prolonged PTT, normal platelet levels
what’s the tx for hemophilia A?
Factor 8 infusion
Desmopressin (DDAVP) - transiently increases factor 8 & vWF release (used prior to procedures to prevent bleeding)
when is Desmopressin used in hemophilia A?
prior to procedures to prevent bleeding (b/c transiently increases factor 8 & vWF release)
what is hemophilia B?
x-linked recessive trait
lack of factor 9
how do you dx hemophilia B?
decreased serum factor 9, prolonged PTT
what’s the tx for hemophilia B?
factor 9 infusion (can’t use DDVAP)
what hemophilia can you use Desmopressin in? what other d/o is it the tx for?
hemophilia A
tx for vWF disease
what are the s/s of vWF disease?
***Mucocutaneous bleeding: easy bruising, epistaxis, gums, GI menorrhage, bleeding after minor lacerations
how do you dx vWF disease?
decreased vWF levels, bleeding time & PTT prolongation worse with aspirin
***decreased Ristocetin activity test = GOLD STANDARD
what’s the GOLD STANDARD to dx vWF disease?
Ristocetin activity test
- will be decreased
- no pat aggregation w/ Ristocetin (ristocetin is an abx that causes pat agglutination in vitro)
what’s the tx for vWF disease?
Type 1 - quantitative def. (MC type)
- mild -> no tx
- mod -> DDAVP (Desmopressin)
Type 2 (qualitative def) -> DDAVP (vWF + Factor 8 prior to procedures)
what should be given to vWF disease and hemophilia b prior to procedures?
DDAVP (desmopressin)
what’s vWF disease?
ineffective plt adhesion -> d/t deficient/defective vWF
what cells are pathognomonic to Hodgkin’s Lymphoma?
Reed-Sternberg cells (“owl-eye” appearance)
how do you dx Hodgkin’s lymphoma?
Excisional bx of LNs (LNs are painless)
-see Reed-Sternberg cells
Mediastinal lymphadenopathy
what are s/s of Hodgkin’s lymphoma?
- Painless lymphadenopathy - alcohol may induce LN pain
- Systemic “B” symptoms in advanced disease (night sweats, weight loss, cyclical fever - Pel-Ebstein fever)
Mediastinal lymphadenopathy
what LNs are MC affected in Non-Hodgkin’s lymphoma?
peripheral LNs
what’s the s/s of NHL?
painless lymphadenopathy, extra nodal sites common (GI, skin & CNS)
what’s the tx for NHL? (follicular and diffuse Large B cell)?
Follicular: Rituximab
Diffuse Large B cell: MC type & most aggressive type. treat with R-CHOP (chemo)
what is the Philadelphia chromosome a/w?
CML (chronic myelogenous leukemia)
what are Auer rods a/w?
AML
what is TTP (thrombotic thrombocytopenia purpura)?
Pentad:
- Thrombocytopenia (petechiae, mucocutaneoux bleeding)
- Microangiopathic hemolytic anemia (anemia, jaundice, schistocyts)
- kidney failure/uremia
- neurologic sx’s (HA, CVA, AMS)
- fever
what are the causes of TTP?
Primary: idiopathic (autoimmune) -> antibodies against ADAMTS13 -> decr. ADAMTS13
what does def. in ADAMTS13 in TTP mean?
small vessel thrombosis -> hemolytic anemia
what’s the dx for TTP?
Labs: thrombocytopenia, NORMAL COAGS (PT/PTT)
what’s the TOC for TTP? other tx?
Plasmapheresis = TOC
Corticosteroids = other tx
what is s/s of DIC?
widespread hemorrhage: venipuncture sites
thrombosis, gangrene
what’s the dx for DIC?
Incr thrombin formation:
-decr. fibrinogen, incr. PTT/PT/INR, severe thrombocytopenia
Increased fibrinolysis: incr. D-dimer
what’s the tx for DIC?
treat the underlying cause (most impt. step in tx of DIC)
FFP if severe bleeding (replaces coag factors)
what is ITP?
idiopathic autoimmune thrombocytopenia purpura
isolated thrombocytopenia (low plt count)
who is acute ITP vs chronic ITP most common in?
acute ITP: MC in children after a viral infection (self-limited)
chronic ITP: MC in adults (recurrent)
what’s the cause of ITP?
autoimmune antibody rxn vs. plts*** -> with splenic plt destruction following acute infection (ex. viral)
what’s the s/s of ITP?
increased mucocutaneoux bleeding: purpura, petechiae, bullae, epistaxis (NO SPLENOMEGALY)
what’s the dx of ITP?
isolated thrombocytopenia with normal coag tests
what’s the tx for ITP (children vs adults)
children: observation (usu. resolves)
adults: corticosteroids (blunts the immune response) -> IVIG -> splenectomy if refractory
what’s the tx after gastric or bariatric surgery if iron deficient?
IV iron b/c of inadequate absorption
what’s the indications for IV iron repletion?
ongoing blood loss, issues with absorption, intolerable GI side effect with PO iron
what’s the indications for PO iron repletion?
not good absorption for PO with prior bariatric surgery
increased GI adrs
easy to use
