6/12 UWorld Flashcards
1
Q
What are the cartilage, muscle, and nerve derivatives of the 1st branchial arch?
A
- Cartilage:
- Maxillary process, mandibular process, incus, malleus
- Muscles:
- Muscles of mastication, mylohyoid, tensor tympani, tensor veli palatine
- Nerves:
- CN V2 and V3
2
Q
What are the cartilage, muscle, and nerve derivatives of the 2nd branchial arch?
A
- Cartilage:
- Stapes, styloid, stylohyoid
- Muscles:
- Muscles of facial expression
- Stapedius, stylohyoid
- Nerves:
- CN VII
3
Q
What are the cartilage, muscle, and nerve derivatives of the 3rd branchial arch?
A
- Cartilage:
- Greater horn of hyoid
- Muscles:
- Stylopharyngeus
- Nerves:
- CN IX
4
Q
What are the cartilage, muscle, and nerve derivatives of the 4-6th branchial arch?
A
- Cartilage
- Arytenoids, cricoid, thyroid
- Muscles:
- 4th à Cricothyroid, most pharyngeal constrictors, levator veli palatini
- 6th à All laryngeal muscles except cricothyroid
- Nerves:
- 4th à CN X superior laryngeal branch (swallowing)
- 6th à CN X recurrent laryngeal branch (speaking)
5
Q
Antibiotic associated with serotonin syndrome
A
Linezolid
MOA - inihibits 50S ribosomal subunit
Also has MAOI action
6
Q
Differences between lesions in the right vs. left frontal lobe
A
Deficits in executive functioning, concentration, orientation, judgment, personality
Left sided lesions – associated with apathy and depression
Right sided lesions – associated with disinhibited behavior
7
Q
What is HELLP syndrome
A
- Stands for:
- H = Hemolysis (anemia)
- EL = Elevated Liver enzymes (RUQ pain, jaundice)
- LP = Low Platelets (bruising bleeding)
- Preeclampsia + thrombotic microangiopathy
- Hemolysis = thrombi causing schistocytes
- Liver enzymes = lack of RBCs leads to infarction of liver tissue
- Platelets = all used up in thrombi
8
Q
MOA of Eptifibatide
A
-
GP2b/3a antagonists
- Monoclonal IgG antibody that binds GP2b/3a receptors, preventing platelet aggregation
- Sketchy = Tied game
9
Q
MOA of Ticagrelor
A
- ADP receptor antagonist – Blocks platelet ADP surface receptors (P2-Y12 receptor blocker) - prevents expression of G2b/3a and thus prevents platelet aggregation
- Sketchy: hot dog grill
10
Q
MOA of Tirofiban
A
GP2b/3a antagonists
Monoclonal IgG antibody that binds GP2b/3a receptors, preventing platelet aggregation
Sketchy = Tied game
11
Q
MOA of Abciximab
A
GP2b/3a antagonists
Monoclonal IgG antibody that binds GP2b/3a receptors, preventing platelet aggregation
Sketchy = Tied game
12
Q
What is agranulocytosis
A
Usually refers to low neutrophils
Increased risk of infection
13
Q
What are the names of the phosphodiesterase inhibitors involved in inhibiting platelet aggregation
MOA?
A
-
Phosphodiesterase inhibitors à “Don’t PHOSTER DISINTEREST in sports” sign
- Cilostazol = lost the ball
- Dipyridamole = two pyramids as top of tent
- MOA:
- Inhibition of phosphodiesterase leads to increases cAMP within the platelet which will activate protein kinase A, and impairing platelet function and aggregation
14
Q
Lab findings (BT, PT, PTT) + treatment in von Willebrand disease
A
- Genetic vWF deficiency
- Findings:
- Increased bleeding time – decreased platelet adhesion
- Increased PTT – vWF normally stabilizes Factor VIII
- Normal PT
- Treatment
- Desmopressin – increases vWF release from Weibel-Palade bodies of endothelial cells
15
Q
Lab findings in DIC
A
- Increased bleeding time – low platelets
- Increased PT and PTT
- Low fibrinogen – being used up
- High D-dimer (fibrin split products)
- Schistocytes
16
Q
Describe the cause + treatment of immune thrombocytopenia
A
- IgG autoantibodies to GP2b3a
- Antibodies produced by plasma cells of spleen and antibody-bound platelets consumed by macrophages of spleen
- Treatment = splenectomy
17
Q
Describe the defect in Thrombotic thrombocytopenic purpura (TTP)
A
- Platelets used up in pathologic formation of microthrombi in small vessels
- Due to decreased ADAMTS13, enzyme that normally cleaves vWF for degradation
- No vWF degradation = abnormal platelet adhesion = microthrombi
18
Q
Findings in TTP vs. HUS
A
- TTP:
- Findings (Pentad):
- Thrombocytopenia = platelets being used up
- Microangiopathic hemolytic anemia = RBCs sheared by microthrombi
- Renal insufficiency (thrombi involve vessels of the kidney)
- Neurological symptoms (confusion, HA, seizures, coma) – thrombi involve vessels of CNS
- Fever
- Findings (Pentad):
- HUS:
- Findings (triad):
- Microangiopathic hemolytic anemia
- Renal insufficiency (thrombi involve vessels of the kidney)
- Thrombocytopenia
- Findings (triad):
19
Q
Compare the defect in Bernard-Soulier syndrome vs. Glanzmann thrombasthenia
A
- Bernard-Soulier syndrome
- Defect of glycoprotein 1b
- Platelet can’t bind to vWF on collagen = defect of platelet plug formation
- Platelet count is only slightly low – moderate thrombocytopenia
- Glanzmann thrombasthenia
- Genetic GP2b3a deficiency
- Defect in platelet aggregation
- Platelet count is normal (they aren’t being destroyed, just can’t aggregate)
20
Q
Describe the basic difference between Hodgkin and Non-Hodgkin lymphoma
A
Hodgkin - Rare neoplastic cells (Reed-Sternberg cells) which secrete cytokine that draw in inflammatory cells, leading to a mass
Non-Hodgkin - Whole mass composed of malignant cells
21
Q
What are the surface markers of Reed Sternberg cells
A
- Large cells with multiple/bi-lobed nuclei with clearing around the nuclei (“owl-eye”)
- RS cells are CD15+ and CD30+
- THINK: 2 owl eyes x 15 = 30
22
Q
Which lymphoma is more associated with B symptoms
A
Hodgkin - Due to cytokine released by RS cells
23
Q
Most common type of Hodgkin lymphoma
A
Nodular sclerosing type
24
Q
Hodgkin lymphoma with best prognosis
A
Lymphocyte-predominant
25
Describe age distribution of Hodgkin vs. Non-hodgkin lymphoma
* Hodgkin - bimodal distribution (peak at 20 and 65 y/o)
* Think of 2 owl eyes as 2 humps
* NHL - variable age range
26
What is the translocation in follicular lymphoma
* t(14;18) = heavy chain Ig (14) and BCL-2 (18)
* Overexpression of BCL-2 = decreased apoptosis
* Recall that BCL2 stabilizes mitochondrial membranes, preventing leakage of cytochrome C, and thus preventing apoptosis
* Follicle is where B-cells are produced, so lack of apoptosis = malignant B-cell proliferation
* THNK: 18 y/o is when you can buy a gun and start killing people (apoptosis)
* Apoptosis occurs in the follicle
27
What lymphoma presents with "starry sky" histology
Burkitt lymphoma
* “Starry sky” appearance – sheet of lymphocytes with interspersed “tingible body” macrophages
* THINK: Burkitt = Africa - sleeping under the stars
28
What is small lymphocytic lymphoma
Lymphoma equivalent of CLL
SLL/CLL
29
What diseases are associated with marginal zone lymphoma
* Associated with chronic inflammatory states:
* Sjogren syndrome
* Hashimoto thyroiditis
* H. pylori
* Marginal zone = area surround the mantle zone
* Marginal zone is present when the cells in germinal center are activated (i.e. inflammation), so it makes sense that this lymphoma occurs in chronic inflammatory states
30
What is Sezary syndrome and what do Sezary cells look like?
* If malignant T-cells of Mycosis fungoides leave the skin lesions and get into the blood it is called Sezary Syndrome
* Sezary cells = lymphocytes with cerebriform nuclei on blood smear
31
What serum markers indicate ALL
* TdT (+) and PAS (+)
32
Translocation and surface marker associated with B-ALL
* CD10 (+)
* t(12;21) = better prognosis
* t(9;22) = poor prognosis
33
Markers and histology associated with AML
* MPO (+) and PAS (+)
* Auer rods
* Crystal aggregates of MPO
* Especially seen in Acute promyelocytic leukemia, AML
34
Common presentation of AML
* DIC is a common presentation
* Auer rods activate coagulation cascade
35
Translocation of AML
* t(15;17)
* Translocation of retinoic acid receptor
* Think:
* Auer sounds like Fuer (Hitler) – Sound of music
* I am 16 going on 17 (15;17)
* Poke Hitler in the eye with a carrot – treat with retinoic acid
36
Common surface markers + histology of CLL
* 95% have B-cell markers (rather than T-cell) à CD20+ and CD5+
* Characteristic smudge cells
* THINK: CLL = Crushed Little Lymphocytes = smudge cells
37
What is the Richter transformation
* Progression of CLL to aggressive lymphoma, usually diffuse large B-cell lymphoma
38
Complications of CLL
Recall that CLL involves mostly B-cells
* Hypogammaglobulinemia
* Neoplastic B-cells don’t make Ig
* May cause autoimmune hemolytic anemia (both warm of cold cell agglutinins)
* If neoplastic B-cells do make Ig, it will be defective and can attack the body
* Richter transformation
* Progression to aggressive lymphoma, usually diffuse large B-cell lymphoma
39
What are the myeloproliferative disorders
Aka chronic leukemias of myeloid cells:
- Chronic myeloid leukemia (CML)
- Polycythemia vera
- Essential thrombocythemia
- Myelofibrosis
40
Compare progression of CLL to progression of CML
CLL may progress to diffuse B-cell lymphoma (Richter transformation)
CML may progress to AML (80%) or ALL (20%)
41
Treatment of CML
* Responds to Imatinib (bcr-able tyrosine kinase inhibitor)
42
Mutation and presentation of polycythemia vera
* JAK2 kinase mutation
* Increased red cell mass, without elevated EPO
* Presentation:
* Plethora (flushed face)
* Blurred vision and HA (due to hyperviscosity)
* Thrombosis (e.g Budd Chiari)
* Itching after bathing
* Erythromelalgia (severe, burning pain and red-blue coloration in the extremities)
43
Mutation and presentation of essential thrombacythemia
* JAK2 kinase mutation
* Presentation:
* Increased bleeding – dysfunctional platelets
* Thrombosis – too many platelets
44
What disorder presents with a "dry tap" on bone marrow aspiration
Myelofibrosis
Hairy cell leukemia (subtype of CLL that causes marrow fibrosis)
45
Mutation in myelofibrosis
JAK2 kinase
All myeloproliferative disorders (polycythemia vera, essential thrombocythemia, myelofibrosis) except for CML have JAK2 kinase mutation
46
Describe the problem in myelodysplastic syndrome
* Defect in cell maturation of all non-lymphoid lineages
* Stem-cell disorder and dysplasia involving ineffective hematopoiesis
* Cause by de novo mutations or environmental exposures
47
Histology of myelodysplastic syndrome
* Pseudo-Pelger-Huet anomaly
* Neutrophils with bilobed nuclei, connected by a thin strand
* Typically seen after chemotherapy
48
What disorders present with lytic bone lesions?
Multiple myeloma
Langerhans cell histiocytosis
Adult T-cell lymphoma
49
Describe multiple myeloma and its classic presentation
* Malignant proliferation of monoclonal plasma cell within the marrow
* Recall: Plasma cells = mature B-cells that produce immunoglobulin
* Presentation:
* Anemia – plasma cells packed in bone marrow inhibit production of other cells
* Rouleaux formation of RBC
* Renal insufficiency – excessive antibodies plug up kidney and form casts
* Lytic bone lesions - plasma cells activate RANK
* Back pain – plasma cells stimulate osteoclasts
* Hypercalcemia – plasma cells stimulate osteoclasts
* M spike
* Increased risk of infection (monoclonal antibodies lack antigenic diversity)
50
Findings in Waldenstrom macroglobulinemia
* B-cell lymphoma with monoclonal IgM production
* Findings:
* M spike due to increased IgM
* Hyperviscosity:
* Blurred vision
* Raynoud phenomenon
* Amyloidosis
* No lytic bone lesions (vs. multiple myeloma)
51
Describe predominant antibodies produced in multiple myeloma vs. Waldenstrom macroglobulinemia
Multiple myeloma = IgG and IgA
Waldenstrom macroglobulinemia = IgM (thing MACRO = IgM)
52
Describe presentation of monoclonal gammopathy of undetermined significance (MGUS)
* Monocloncal proliferation of plasma cells
* Production of monoclonal immunoglobulins = M spike
* No symptoms
* 1-2% progress to multiple myeloma
53
Presentation of Langerhans cell histiocytosis
* Neoplastic proliferation of specialized dendritic cells (especially in skin)
* Birbeck (“tennis racket”) granules seen on EM
* S-100 (+) and CD1a (+)
* Presentation:
* In children
* Lytic bone lesions
* Skin rash
* Recurrent otitis media
54
What is the effect of the t(9;22) translocation seen in CML
BCR-ABL
Constitutively active tyrosine kinase)
