PPP ROSH- Psych/Heme Flashcards
Polycythemia Vera’s presentation
This is d/t ? mutation
What abnormal c/c can they have
Splenomegaly
Abnormal proliferation of all 3 lines
Thrombosis
JAK-2
Aquagenic pruritus: pruritus after warm bath
How is Polycythemia Vera Dx and Tx
MC nutritional deficiency in Peds
? is the MC inherited bleeding d/o
CBC; Hydroxyurea, ASA, Phlebotomy
Fe deficiency
VwF- supports platelet adhesion by bridging platelet w/ endothelial structures
How do Pts w/ VW Dz present
How are Pts Tx
MC hemolytic anemia
Mucosal bleeds
Desmopressin- DDAVP
Hereditary spherocytosis- RBC membrane defect
What lab results are seen w/ Hereditary Spherocytosis
What is the MC complication from this Dx
What characteristics differentiates TRALI from TACO
Howell Jolly body
Billirubin gallstones
TACO has no fever, TALI- Fever, HOTN, Tachycardia
Reed Sternberg cells indicate ? Dx
What is the MC nephrotic syndrome in Peds
What is the MCC of nephrotic syndrome in adults
HL
Minimal change dz
Focal segmental glomerulosclerosis
What are the B-Sxs of HL
MC inherited hypercoagulability d/o
How are these Pts Tx
Fever, Night sweats, Weight loss
Factor V Leiden- needed to generate fibrin mesh of clots, Dz causes resistance to degradation by Protein C
Anticoagulation, FFP
Name of nail changes seen during Fe Deficient Anemia
? lab result suggests Vit B12 deficiency
What other supplement Tx is recommended during first months of Tx
Koilonychia- spooning of nails
Megaloblastic anemia w/ inc serum methylmalonic/homocysteine
Folic acid
MCC of Megaloblastic anemia
What can cause Methemoglobinemia and how is it Tx
What would be seen on blood draw
Chronic alcoholism: B12 deficiency, Folate deficiency
Benzocaine:
Methylene blue- aids w/ Fe3 (ferric) conversion to Fe2 (ferrous)
Chocolate colored arterial blood
Define Fanconi Anemia
What may be noted on PE
How are Pts Tx
Defected DNA repair causing macrocytic anemia and inc AF-protein; increased risk for AML/tumors
Absent/Small thumbs, Short, Mental delays
Marrow transplant
How are Peds/Adults going through Sickle Crisis induced cerebrovascular events Tx
How are vaso-occlusive pain crisis’ Tx
How are acute chest syndromes Tx
P: Exchange transfusion to dec Hgb S level <30%
A: tPA
Hydroxyurea w/ support
ABX w/ exchange transfusions
How are Sickle Cell induced Aplastic Crisis Tx
How are Sickle Cell induced priapisms Tx
What are the MCC of Acute Chest syndrome in Sickel Pts
P-RBC transfusions
Terbutaline and exchange transfusion
Chlamydia, Mycoplasma
How long after completion of chemo will Nadir in absolute neutrophil count occur
MCC of aplastic crisis in Sickle Cell Pts
Sickle Pts should receive ? type of blood products
5-14d later
Parvovirus B19 d/t suspension of erythropoiesis (undetectable retic count)
Multiple platelet/PRBCs
How are DVTs in pregnancy Tx
What is the name of the blue-ish hue the leg appears as
Best lab test for indicator of stored iron
LMWH/UFH, Warfarin is C/i
Phlegmasia ceruelea dolens
Ferritin
What lab result is used to differ Fe Deficient Anemia from Thalassemia
? type of x-rays are ordered for suspected lead poisoning
What lab result is pathognemonic to this poisoning and how are they Tx
RDW- inc w/ Fe Deficient, normal w/ Thalassemia
Knee/Wrist
Basophilic stippling; Succimer
Sickle Cell Dz affects ? Hgb chain
What microbes are these Pts particularly susceptible to
? is the leading cause of death in these Pts and how are they Tx
B
HFlu, N meningitides, Strep Pneumo
Acute chest syndrome: admit, broad spectrum ABX, O2
What lab result would be seen during B-Thalassemia
What 3 lab results are seen during thalassemia
N-HL presentation
Codocytes: target cells; abnormal B-cahin causes hypochromic appearance
Microcytosis, Hypochromic, Basophilic stippling
Persistent, painless adenopathy w/ B-Sxs
What meds are used pre/acutely for chemo s/e when treating N-HL
Primary Immune Thrombocytopenia is AKA
How are PTs Tx
Pre: Benzos Acute: 5-hydroxytryptamine antagonist
Idioapthic Thrombocytopenia Purpura- Abs to platelets causing mucocutaneous bleeds
CCS+IVIG; <10K= spontaneous bleeds- Platelet transfusion
Define Multiple Myeloma
How do Pts present w/ this
How is this Tx
Malignant proliferation of plasma cells producing excess monoclonal paraproteins
CRAB:
Inc Ca, Renal insufficiency (Bence Jones protein), Anemia, Bone lesion (HyperCa)
CCS, Pamidronate, Chemo
MC leukemia in Western country adults
What may be seen on lab results
Define Tumor Lysis Syndrome
CLL- monoclonal proliferation of mature B-cells
Smudge cells- fragile lymphocytes
>10% clonal marrow plasma cells =Dx
Hyper-K/Uric/Phosph, HypoCa, Inc Cr d/t cell death from chemo for heme malignancies; Tx: aggressive fluids and dialysis
? syndrome can cause multiple spontaneous abortions
This is commonly seen w/ ? Dz
What markers are used for Dx
Antiphospholipid Ab syndrome- acquired hypercoagulability
Lupus
Anticardiolipin Abs, Anti-beta 2 glycoprotein Abs, Lupus anticoagulant
What do blood samples look like under microscopy during Multiple Myeloma
How are PTs Tx
B12 Deficiency will have ? increased lab result
Rouleaux formation
Chemo, Stem cell transplant
Methylmalonic acid
? is the hallmark finding for multiple myeloma
Philadelphia chromosome is found w/ ? Dx
What is the staging system for N/HL
Bence Jones proteins
CML- WBC 150K, peripheral smear w/ L-shifted myeloids, Splenomegaly, <5% blast cells; Tx: Imatinib mesylate
Ann Arbor system
? lab test will be abnormal in Pts w/ hemophilia B
This hemophilia is AKA and d/t ?
How are Pts Tx
aPTT
Christmas Dz d/t deficiency of Factor 9
Recombinant Factor 9
Define TTP
How are Pts Tx
? is the MCC of hyperviscosity syndrome and how are they Tx
Dec VWF cleaving protease leading to inc LDH, indirect bili and normal coag studies
Plasma exchange w/ CCS
Inc serum protein 2/2 Waldenstrom macroglobulinemia; Phoresis/Phlebotomy
? Dx is associated w/ Auer Rods
When is Clozapine used
What lab needs to be ordered when using Clozapine
AML- MC leukemia in adults; clonal proliferation of myeloid precursor cells
Schizo w/ SI
WBC monitoring for agranulocytosis
Define Muehrckes Lines
MCC of non-solid organ Ca-related death
Leading cause of death world wide
Transverse bands that disappear w/ pressure
N-HL
Adeno lung Ca
? is the only dz modifying therapy for Sickle Cell
MCC of hemolytic transfusion reactions
How are these Tx
Hydroxyurea- inc levels of Hb F (fetal hgb)
ABO incompatability d/t human error
Stop infusion, Crystalloid infusions, Diuretics
ALL
AML
CLL
CML
MC childhood leukemia
Adults > kids w/ Auer rods
MC adult leukemia w/ smudge cells; worst prognosis
Philadelphia chromosome w/ basophilic smears
How can CML be differed from reactive leukocytosis d/t infection
2nd Gen antipsychs need to have ? monitoring
Cluster A, B, and C personalities
CML- Phili chromosome
RL: lower WBC <50K and w/out splenomegaly
Lipids d/t acquired metabolic syndrome
A: Schizotypal, Schizoid, Paranoid
B: Boderline Antisocial Histrionic Narcissistic
C: Dependent, OCD, Avoidant
C/i for using Buproprion
? presenting c/c suggest panic attack
How is PCP use differed on PE from cocaine
Anorexia nervosa, Seizure d/o
Chest pain
PCP- aggressive/violent behavior or flat, catatonic stare
Opiate withdrawal Sxs
How is GAD/o Tx
Blocking ? receptors decreases risk for EPS
Mydriasis- dilated
N/V/D
Tachy/Tachy/HTN
Yawning/lacrimation
Venlafaxine (SNRI)
Muscarinic Ach receptors
What med can be used in Anorexia Nervosa to aid w/ weight gain
Pts w/ delusional parasitosis can present w/ ? sign
First line therapy for borderline personality d/o
Olanazpine- atypical antipsych
Matchbox- sample of skin/infestation
CBT w/ psychotherapy
Define Kaposi Sarcoma
MC head/neck Ca in Peds
What lab result aids w/ Dx
Systemic tumor d/t co-infection w/ HHV-8; presents w/ ecchymotic macules/tan plaques in Eastern European males w/ purple/brown lesions on feet
HL- painless, rubbery mass in supraclavicular/anterior cervical triangle w/ B-Sxs
Reed Sternberg cells
? virus is associated w/ Burkitt Lymphoma
How does Hereditary Spherocytosis present and how is it Tx
Unique fact to isolate this Dx
EBV
Spherical microcytic, Inc retic/MCV, +osmotic fragility test, -Coombs; Tx: Splenectomy
Inc mean corpuscular hgb concentration (MCHC)
What RBC membrane protein is defected in spherocytosis
Define HIT
How is it Tx
Sprectrin or Actrin
Heparin Induced Thrombocytopenia- 5-7d post initiation drop in platelets >50%
D/c heparin, start Fondaparinux, FXa inhibitors
Platelet levels below ? should initiate transfusions
MC form of inherited Aplastic Anemia
Infants drinking cows mild before ? age are at risk for Fe Anemia and what is Tx
<10K
Fanconi Anemia
<12mon; Ferrous sulfate 2mon after Hgb normalizes
Name of triad contributors to thrombosis development
Population for the pentad presentations for TTP and HUS
What will be seen on PE
Virchow: Stasis Hypercoag Injury
HUS: kids, TTP: adults
Fever Anemia Thrombocytopenia Neuro Kidney injury
Purpura, Petechiae, Bleeding
How is TTP Tx
MC hereditary bleeding d/o
3 common findings for clotting factor deficiency and not platelet insufficiency
Plasmapheresis- remove plasma, replace w/ FFP
Von Willebrand dz- Tx w/ DDAVP
Delayed bleeding, Hemearthrosis, Soft tissue hematomas
What PE finding is most consistent w/ thrombocytopenia
MCC of Secondary Polycythemia
MC location for spontaneous bleeds d/t hemophilia A
Wet purpura- occurs on mucosal surfaces
Hypoxia
Peds: Ankle, Adult: Knee > Elbow > Ankle
Anesthetic allergy and next option algorithm
Max dose of Lidocaine w/out Epi
What is found in cryoprecipitate
Amide: have two I’s, Esters: have one I; allergy to one, pick from other class
3-5mg/kg
Factor 8, 9, vWF, fibrinogen, fibronectin
Define Neutropenic Fever
How is this Dx
How are Pts Tx
Fever w/ neutropenia (absolute neutrophils) <1500 MC d/t Pseudomonas
Blood and Urine cultures
Vanc + Cefepime (4th Gen)
Lab result characteristic for Fe Deficient Anemia
Alpha-Thalassemia is associted w/ ? ancestry
What medication is started in Peds at age 5 w/ Dx of Sickle Cell
RDW elevation
South Asia/Chinese
PCN
Antiplatelets
Anticoagulants
What temp defines a neutropenic fever
Clopidogrel ASA Prasugrel
Dabi/Edox/Apixa/Riva-aban, Warfarin
PO temp of 101* taken once
Warfarin inactivates ? factors
Reversal agent is ?
MCC of bacterial infection in neutropenic fever Pts
2 7 9 10, Protein C, S
Vit K
Gram pos
How is antiphospholipid syndrome tested for Dx
What is used for primary thrombotic prevention
Most Pts are put on life long ? for anticoagulation
Lupus anticoagulant, Anti-cardiolipin
Low dose ASA
Warfarin
What med is used for prophylactic Tx for Pts w/ antiphospholipid syndrome and SLE
Hereditary spherocytosis are at increased risk for infections by ?
What med is used for Pts w/ hemophilia prior to dental procedures to prevent clot breakdown
Hydroxychloroquine
Parvovirus B19
Aminocaproic acid
? types of antipsychotics are more likely to have EPS Sxs
What med is used for medical Tx failure, chronic and recurrent urticaria
Howel Jolly bodies may be seen in ? population w/ ? Dx
Typicals
Doxepin- TCA
Omalizumab
PO Antihistamines
Post exertion/head in Sickle Cell Pts
What do Howel Jolly bodies look like
What does their presence indicate
What will ALL look like on PE
Basophilic nuclear remnant in RBCs
Damage/absent spleen
Peds 3-7y/o w/ bone pain, adenopathy, hepatosplenomegaly and CNS involvement, lymphoblasts on peripheral smear
Initial lab test for Dx B12 deficiency
MC presentation for ALL
How is a Dx of Multiply Myeloma confirmed
Serum cobalamin
Adenopathy
Serum/Urine protein electrophoresis
How is a Dx of TTP confirmed
How are PTs Tx
What lab results are seen w/ vWDz
ADAMTS13 deficiency w/ inhibitor (cleaves vWF)
Plasma exchange (platelets <10K), Rituximab, GCCS
Prolonged bleeding time; Normal PT, PTT, INR
How is an anaphylactoid reaction to Vanc Tx
Alcoholic w/ generalized weakness needs ? supplementation
Alcoholics are commonly deficient in ? but will have ? associated lab result
D/c infusion, Push diphenhydramine
Folic Acid
Thiamine w/ no anemia
B12 deficiency can be associated w/ ? infection
Philadelphia chromosome is associated w/ ? Dx and w/ ? gene
How to differ Fe Deficient, ACDz, and Thalassemia
D latum tapeworm
CML, BCR-ABL gene
Fe: micro/hypo w/ dec ferritin, inc TIBC
ACDz: inc ferritin, dec TIBC
Thal: norm ferritin, norm/inc serum Fe
How to differ aplastic crisis from splenic sequestration
How to differ Primary/Secondary Erythroctyosis (polycythemia vera)
Opposition defiant d/o commonly co-exists w/ ? other Dx
Aplastic; dec retic, mild neutropenia
Sequest: splenomegaly, hypovolemia, thrombocytopenia
1: normal o2 sat, dec EPO, inc WBC/platelet
2: dec o2 sats, inc EPO, normal WBC/platelet
ADHD
