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