amboss 6/25 Flashcards
what are some hereditary causes of hyper coagulability
factor V leiden, protein C def, protein S def, antithrombin III def, hyperhomocysteinemia, plasminogen def, sickle cell anemia
what are some acquired causes of hypercoagulability
pregnancy, age, smoking, obesity, surgery, immobilization, trauma, malignancy, nephrotic syndrome oral contraceptive use, SLE, heparin-induced thrombocytopenia
what is the result of heparin treatment
increases the PTT; X inhibitor
what is the differential in someone that is treated with heparin, but their PTT doesnt change
this means that they are heparin resistant. Usually antithrombin III, high levels of VIII or fibrinogen
what does the PTT measure; which factors
VII, XI, IX, VIII, X V, II, I
what would you expect to find on peripheral smear from someone with sickle cell anemia
holly-jolly bodies
sickled cells
what are people with sickle cell trait at risk of developing
renal papillary necrosis
what GI disorder are people with sickle cell disease at risk for
cholelithiasis
what are the most common presentation of people with sickle cell trait
hyposthenuria (lots of urine with low osmolarity due to kidney dysfunction). painless hematuria as well. UTIs, chronic kidney disease, renal medullary carcinoma.
what is the best treatment for sickle cell
hydroxurea becuase it reduces the incidence of vasoocclusive crises
what are the mechanisms of hydroxyurea treatment for sickle cell
increases reticulocyte and fetal hemoglobin
what is the presentation of paroxysmal nocturnal hemaglobinuria
intermittent abdominal pain, jaundice, hemaglobin in the urine, intravascular hemolysis with normal blood smear and combs test because this is complement mediated, not antibody. they will have dark urine in the morning
what does paroxysmal nocturnal hemaglobinuria put the patient at highest risk for
thrombosis; this is the most common cause of death
what is the presentation of intravascular hemolysis
increased LDH, reticulocytes, hemoglobinuria, and decreased haptoglobin
What is the cause of anemia in an elderly person with a murmur in the right upper field
aortic stenosis causing shearing forces on the RBC
what is the cause of thrombocytopenia in cirrhosis
The main cause is cirrhosis causes portal hypertension and decreased venous drainage from the spleen. This causes hypersplenism and sequestration of upwards of 90% of platelets in the spleen.
also, due to liver dysfunction there is a paucity of hormones (thrombopoeitin) for the production of platelets
what is the presentation of chronic myelogenous leukemia
fatigue, splenomegaly and SEVERE leukocytosis (250,000)
what is the presentation of acute myelogenous leukemia
fatigue and splenomegaly with leukocytosis (80,000), with Auer rods (cytoplasmic inclusion needles)
what is the significance of 9:22 translocation
this is the Philadelphia chromosome BCR:ABL acute lymphocytic leukemia.
what is the first line therapy for acute myelogenous leukemia
imatinib
what is the definitive diagnostic test for leukemia
bone marrow biopsy; a blood smear is not definitive
what are Down syndrome patients at risk of developing for blood cancers
acute lymphoblastic leukemia, acute myeloid leukemia
what is the best way to prevent tumor lysis syndrome
IV hydration
presentation of pediatric HUS
This child presents with thrombocytopenia, signs of hemolysis (normocytic anemia, ↑ LDH, indirect hyperbilirubinemia, schistocytes), and renal dysfunction (hematuria, proteinuria, ↑ BUN, and ↑ creatinine), which is a triad indicative of hemolytic uremic syndrome (HUS).
most likely cause of pediatric HUS
E. coli (STEC) is the most common cause of pediatric HUS (90% of cases)
what is the treatment fro HUS
hemodialysis, supportive care.
what are the indications for hemodialysis for hUS
oliguria/anuria, azotemia, hyperkalemia, and acidosis. This patient with acidosis and oliguria should be started on hemodialysis in addition to supportive
is plasma exchange used in HUS
rarely. it is not very effective, but can be used in refractory cases.
what is the triad of HUS
thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney injury
what is the best prophylactic for malaria in a pregnant woman
mefloquine
what is the staining for acute myeloid leukemia
CD33/34 and myeloperoxidase
what antibody do we test for in antiphospholipid
anticardiolipin; lupus anticoagulant
what is the presentation and treatment for acute pyogenic lymphadenitis
usually a swollen, tender, progressively enlarging lymph node on the side of the neck. treat with clindamycin
what is the presentation of splenic sequestration crisis
REMEMBER YOUNG CHILD; PRIOR TO AUTOSPLENECTOMY. reticulocyte count increases, hypovolemia, acute onset severe anemia.
what is the presentation of immune thrombocytopenia
isolated thrombocytopenia caused by IgG against platelets. women of child bearing age. bleeding from the gums, petechia and purpura.
do we give platelet transfusions to patients with ITP
no. they are rapidly destroyed upon infusion
what is the treatment for ITP
usually observation and they typically spontaneously resolve within 6 months
does bleeding occur in thrombocyopenia
rarely. even with severe <30,000.
what are the treatments for ITP
corticosteroids, IVIG, and splenectomy
what is the presentation of hemophilia
normal bleeding time, normal platelets, history of easy bruising and bleeding into joint spaces. bleeding with surgeries. typically a family history of bleeding disorders
who is affected by hemophilia
males
what parameter is prolonged in hemophilia
PTT
what is the presentation of vonwillebrands
increased PTT (can be normal), increased bleeding time. inherited autosomal dominant
what is given for warfarin reversal for emergent surgery
vitamin K and fresh frozen plasma
what is the most specific test for hereditary spherocytpsis
Eosin-5-meleimide test.
what tests are there for hereditary spherocytosis
eosin-5-meleimide test, osmotic fragility test
what are the therapies for hereditary spherocuytosis
transfusions and phototherapy for acute hemolysis, folic acid supplementation, splenectomy
what is a side effect of TMP-SMX
agranulocytosis. neutropenia/neutropenic fever
what is the most serious side effect of carbamazepine usage
agranulocytosis and aplastic anemia
what is the treatment for beta thalassemia minor
nothing
what is beta thalassemia minor
Minor variant (heterozygous): unremarkable symptoms (low risk of hemolysis, rarely splenomegaly)
what is beta thalassemia major
Absence of beta-globin. Major variant (homozygous)
Severe hemolytic anemia that often requires transfusions → secondary iron overload due to hemolysis, transfusion, or both → secondary hemochromatosis
Hepatosplenomegaly
Growth retardation
Skeletal deformities (high forehead, prominent zygomatic bones, and maxilla)
Transient aplastic crisis (secondary to infection with parvovirus B19)
usually presents in childhood.
what is alpha thalassemia silent carrier
asymptomatic
what is alpha thalassemia trait
mild hemolytic anemia with normal RBC and RDW
what is hemoglobin H disease
Hemoglobin H disease
Jaundice and anemia at birth
Chronic hemolytic anemia that may require transfusions → secondary iron overload due to hemolysis, transfusion, or both → secondary hemochromatosis
Hepatosplenomegaly
Skeletal deformities (less common)
Compared to thalassemia beta, symptoms in adults are generally less severe.
what is HbBarts
Hb-Bart’s hydrops fetalis syndrome (most severe variant of alpha thalassemia)
Intrauterine ascites and hydrops fetalis, severe hepatosplenomegaly, and often cardiac and skeletal anomalies
Incompatible with life (death in utero or shortly after birth)
what is alpha thalassemia minima
silent carrier with normal HbA, A2, HbF, and HbH and HbBarts are absent.
what is alpha thalassemia minor
this is trait. decreased HbA, A2, and HbF, increased HbH, but absent HbBarts
what is alpha thalassemia intermediate t
HbH disease. two alleles of HbA and A2 as well as HbF greatly diminished. two HbH and one Hb Barts
what is Hb Barts disease
this is major. HbA and A2 as well as HbF absent. two HbH and two Hb Barts
when do sickle cell patients undergo asplenia
by the age of 4
what empiric treatment should be given to sickle cell patients who present with fever and possible sepsis (over the age of 4)
broad spectrum antibiotics such as ceftriaxone for coverage of gram positive and negative bacteria
staph, strep, salmonella and e coli are all common in sickle cell disease
what are patients with spherocytosis at risk for and what can reduce this risk
gallstones. cholecystitis.
splenectomy
what are patients with thalassemia at risk for
skeletal deformities
when do you expect antibiotics to cause anemia in G6PD deficiency
within 2-3 days
what is the hallmark of spherocytosis
increased RDW and MCHC you asshole!
what is the hallmark of spherocytosis
increased RDW and MCHC you asshole!!
what is the hallmark of spherocytosis
increased RDW and MCHC you asshole!!!
what is the underlying cause of hereditary spherocytosis
defective spectrin on the RBC surface
what are the labs for iron deficiency anemia
decreased iron, decreased ferritin, increased TIBC and RDW, low MCV,
what are the staining for acute lymphoblastic leukemia
terminal deoxynucleotidyl transferase TdT, CD10, CD19
what is the most frequent malignant disease in children and how does it presnet
Acute lymphoblastic leukemia (ALL) is the most frequent malignant disease in children and has a peak incidence between the ages of 2–5 years. It manifests with bone pain, fatigue, fever, hepatosplenomegaly, anemia, thrombocytopenia (with bleeding diathesis), and leukocytosis.
what cell type is responsible for ALL
B cell precursors
what should you think when facing bleeding disorder in a woman
von willebrand
what is the treatment for von willebrand
desmopressin
what is the mechanism of action for von willebrands treatment with desmopressin
increased release from endothelial cell
what can maternal diabetes do to the babies hematologic system
cause polycythemia/hyperviscosity and infarction
what should be supplemented in spherocytosis
folic acid
what does folic acid deficiency look like
glossitis, low reticulocyte count, macrocytic anemia
what is the reason that OCPs cause DVTs
estrogen in the pill decreases protein S levels, which typically inihibit pro coagulable factors
what do protein C and S do? what is the consequence if they do not function
inihibit pro coagulable factors
thrombus formation
what is the underlying cause of G6PD
absence of reduced glutathione
what is the protein responsible for spherocytosis
ankryin
In what leukemia are auer rods the most common
acute promyelocytic leukemia
what is the treatment for acute promyelocytic leukemia
all-trans retinoic acid
what is a characteristic finding for multiple myeloma
rouleaux formation
what is a characteristic finding on histology for chronic lymphocytic leukemia
smudge cells
what is the most common presentation of CLL
generalized lymphadenopathy and splenomegaly in a patient that is over 65.
most common cause of generalized lymphadenopathy
what is the cause of hypercalcemia in Hodgkin lymphoma
increased 1a-hydroxylase activity AKA increased active vitamin D. The patient will have hypercalcemia with LOW PTH, which is not seen in humoral PrTH production.
reed sternberg cells stain how and are pathopneumonic for what
CD15/30 positive
hodgkin lymphom a
what is tartrate resistant acid phosphatase imply
hairy cell leukemia
what are two treatments for hairy cell leukemia
cladribine and pentostatin
what do steroids do to lymphocytes, eosinophils, monocytes and neutrophils
lymphopenia, eosinopenia, decrease in monocyte count, and an increase in neutrophils because it inhibits apoptosis
what is a low alkaline phosphatase score associated with
CML. it is the only cancer that has a low ALKpoH score
what is myelodysplastic syndrome and what is it highly associated with
highly associated with acute myelocytic leukemia
A group of conditions caused by bone marrow failure. Cytopenias (e.g., thrombocytopenia, anemia) are the hallmark. The etiology may be genetic, environmental (e.g., benzene exposure), and/or iatrogenic (e.g., post-radiation myelodysplasia).
what is the presentation for pure red cell aplasia
there will no reticulocytes or very low
what is the presentation of beta thalassemia minor
can present with an isolated microcytic anemia but will be asymptomatic. If there are symptoms look elsewhere
what is the presentation of isoniazid treatment
there will be a microcytic anemia with normal iron levels
what is the treatment for sickle cell stroke
exchange transfusion
do we give tPA to sickle cell patients
no. this is ineffective for sickle cell stroke nd can cause hemorrhage. exchange transfusion protocols are the most effective
