Condition\Diseases

Question 1

Hemolytic transfusion reactions summary

Answer 1

Def: ABO incompability due to human error

SS:

• fever
• flank pain
• red/brown urine

Txt:
medical emergency
stop transfusion
saline 100-200 ml/hr
Vasopressors

Question 2

Febrile nonhemolytic transfusion summary

Answer 2

Def: reaction due to interleukins and TNF alpha substance

SS:
Fever
Chills
Rigors
Mild Dyspnea

Txt:
stop transfusion
Tylenol/benadryl
symptomatic

Question 3

Delayed Hemolytic Transfusion summary

Answer 3

Def: reaction caused by atypical antibody usually 2-10 days after

SS:
slight fever
falling hematocrit
mild increase unconjugated bilirubin
mild increase spherocytes

Txt:
None for this transfusion
future trans: antigen negative blood products

Question 4

Disorders of primary hemostasis

Answer 4

Platelet: quantitative (thrombocytopenia) and qualitatie (functional defects)
Von Willebrand Disease

Question 5

Disorders of secondary hemostasis

Answer 5

Congenital: hemophilia A and B, rare factor deficiences
Acquired: Vit. K deficiency, liver disease

Question 6

von Willebrand disease

Answer 6

Most common inherited bleeding disorder

Def: mutations in the gene coding for von willebrand factor results in dysfunction of platelet adhesion or the dysfunction of carrying of factor VIII and not protecting it

S/S: epistaxis, dental bleeding, menorrhagia, bruising, prolonged bleeding (primary hemostasis), severly affected pts may have joint and soft tissue hemorrhages

Dgx: vW panel: low vW antigen, low factor VIII, low ristocetin cofactor activity (protein doesn’t func); other tests: abnormal platelet func testing, NORMAL INR, aPTT, and platelet count

Tx: avoid drugs that inhibit plt func, DDAVP, antifibrinolytics, plasma derived VIII/vWF, cryoprecipitate

Question 7

Hemophilia

Answer 7

Def: congenital bleeding disorder caused by deficiency of coag factor VIII (type A, classic) or IX (type B, Christmas) (both located on X chromosome)

S/s: clinical phenotypes are indistinguishable; severity of bleeding depends on the factor level; joint, soft tissue, deep muscle, and intracranial bleeding

Dgx: Markedly prolonged PTT, NORMAL INR and plt count and func. measure specific factor levels

Tx: avoid drugs that impair plt func, factor VIII or IX concentrates, prophylactic factor replacement

Question 8

Vitamin K deficiency

Answer 8

Required for the synthesis of coag factors II, VII, IX, and X and protein C and S.
Infants: doesn’t cross placenta, low levels in breast milk, low amount of colonic bacteria
Adults: malnutrition, impaired malabsorption, antibiotics

Question 9

Liver disease

Answer 9

associated with defects in primary and secondary hemostasis and in fibrinolytic system
Thrombocytopenia, decreased synthesis of clotting factors, dysfunctional fibrinogen, excess fibrinolysis
Can cause anticoagulation or coagulation problems because it contributes to both but the fulcrum of the scale is small so it could tip in any direction

Question 10

Venous thromboembolism

Answer 10

occurs in veins
Dx: D-dimer, compression US, v/Q perfusion, CT scan
S/S: pain, swelling, tenderness, discoloration, warmth, asymmetry, dyspnea, chest pain, angina, hemoptysis, cough

Question 11

Genetic thrombophilia

Answer 11

Mechanisms of thrombosis: failure to control thrombin generation (factor V Leiden, prothrombin gene mutation, protein C and S deficiency)
impaired neutralization of thrombin (anti-thrombin deficiency)
Genes + Triggers = clot
Everyone has a thrombosis threshold and genes, triggers, and age bring you closer to that threshold until you hit it and clot
Tx: heparin, life long anti coag

Question 12

Anaphylactic Transfusion Reaction Summary

Answer 12

Def: body develops anaphylactic reaction to transfusion blood products

SS:
shock
hypotension
angioedema
respiratory distress

Txt:
stop transfusion
epinephrine
maintain airway
saline
vasopressors

Question 13

Urticarial Transfusion Reaction Summary

Answer 13

Def: blood products cause histamine release from mast cells and basophils causing hives and urticaria

SS:
hives/urticaria

Txt:
benadryl
continue transfusion

Question 14

Post Transfusion Purpura Summary

Answer 14

Def: sensitazation to foreign antigen from previous platelet transfusion. Very rare. MC - HPA-1a

SS:
severe thrombocytopenia 5 - 10 days post transfusion
Lasts days to weeks

Txt:
high dose corticosteroids
exchange transfusion
subsequent transfusions = washed cells or HPA-1a negative cells

Question 15

Transfusion Related Acute Lung Injury Summary

Answer 15

Def: Rare. Believed to be immune response in pulmonary

SS:
sudden onset respiratory distress during or after transfusion
Fever
tachypnea
tachycardia
HOTN

Txt:
supportive
mechanical ventilation

Question 16

Comps with RH- Preggers

Answer 16

Def:

1. Mother Rh-
2. Has baby1 who is Rh+ and baby1 Rh+ blood get into Mom bloodstream.
3. Nothing bad happen to baby1. Mother now has Rh antibodies in her system
4. Mom get preg again. If have Rh+ baby, her body attacks the fetus and tries to kill it.

Txt:
300 mcg IM Rhogam/fetal blood 15cc during and after preg

Question 17

Splemomegaly Summary

Answer 17

Def: Enlarged spleen

Cause:
Liver dx
Hematologic malignancy
Infection
CML
Lymphoma
Parasitic infection
Gaucher dx

Question 18

Post Splenectomy Sepsis summary

Answer 18

Def: infection via encapsulated bacteria ( s. pneumo, H. flu, N. Men)

Txt:
children - daily prophylaxis until 5 or 3 yrs past surgery
abx filled at home and waiting
- take dose
- arrive at nearest health care facility

Question 19

Anemia (general)

Answer 19

Classifications:
Decreased production: low reticulocyte count, catergorize by red cell size (MCV), abnormalities of WBC and/or platelets
Increased destruction: elevated reticulocyte count, physical exam, lab studies of elevated bilirubin and LD, and low hemoglobin

S/s: fatigue, dyspnea, palpitations, tachycardia, dizziness, lightheadedness, faintness, headache, angina, claudication, pallor, jaundice, scleral icterus, edema

Tests: CBC, reticulocyte count (testing bone marrow function) and smear; MCV to check RBC size ( in children number will be 70 + their age in years til they hit puberty)

Question 20

Microcytic anemia

Answer 20

• Iron deficiency: tx by giving them iron tablets and dgx if it works (adults = colon cancer, chlidren = malnutrition d/t only drinking milk)
• eval: ferritin
• Thalessemia: will see in newborn screening, Hgb electrophoresis
• ACD: CRP and ESR will be elevated
• Lead toxicity: check lead levels

Question 21

Thrombocytosis summary

Answer 21

Def: Too many platelets

Cause:
Myeloproliferative disorders
- essential thrombocythemia
- chronic myelogenous leukemia
- polycythemia vera
Reactive
- infection
- post-splenectomy
- malignancy
- iron deficiency
- inflammation

Question 22

Thrombocytopenia Summary

Answer 22

Def: too few platelets

Cause:
Decrease production
- bone marrow failure/replacement
- marrow toxins
- nutritional deficiency

Increased destruction
- immunologic
drug induced
HIT
ITP
- non-immunologic
DIC
TTP/HUS

Hypersplenism

Question 23

Drug induced Thrombocytopenia summary

Answer 23

myelosuppression

• chemo
• sulfa
• thiazide diuretics
• Valproic acid

immunologic

• antibodies against drug/platelet complex
• penicillin, heparin

Txt:
stop dug
transfuse if necessary
no steroids

Question 24

Heparin induced thrombocytopenia summary

Answer 24

Most important cause of immune mediated thrombocytopenia

Heparin exposure

platelets drop >50% from baseline

Begin 5-10 days after heparin initiated

Life and limb threatening

Variable INR, PTT, fibrinogen

Stop heparin

Question 25

Immune Thrombocytopenia Purpura Summary

Answer 25

Healthy severe thrombocytopenia CBC otherwise normal Normal INR, PTT, fibrinogen Rarely life-threatening ``` SS: Petechiae dependent organs echymosis menorrhagia, epistaxis, gingival bleeding, blood in stool/urine no lymphadenopathy/splenomegaly ``` Eval: CBC normal (low platelets < 10,000) MPV high Normal coag times ``` Txt Adult: Steroids (large dose then taper) IVIG (short efficacy) WinRho Rituxan Splenectomy Observation ``` ``` Txt child: Observe IVIG WinRho Steroids Rituxan Splenectomy ```

Question 26

Disseminated Intravascular Coagulation summary

Answer 26

Sick, bleeding > thrombosis Falling platelets Prolonged INR, PTT, low fibrinogen ``` Cause: infection/sepsis malignancy preg issues severe tissue injury ``` Txt: Treat underlying cause transfuse plateletes, FPP

Question 27

Thrombotic Thrombocytopenic pupura summary

Answer 27

Sick, bleeding and thrombosis, CNS, renal Called Hemolytic uremic syndrome (HUS) in children Falling platelets, MAHA Normal INR, PTT, fibrinogen ``` Cause: Idiopathic Secondary - Preg - Autoimmune - Drug Familial ``` ``` SS: Classic Pentad - Thrombocytopenia - Microangiopathic hemolytic anemia - Neurologic symptoms - Renal dysfunction - Fever Normal coag times ADAMST 13 level ``` Txt: plasmapheresis FFP infusion

Question 28

Normocytic anemia

Answer 28

*where reticulocyte count is important LOW=production problem; kidney failure, bone marrow suppression (virus), bone marrow infiltration (ALL, ASL, infections) HIGH=increase in RBC turnover/destruction -DAT (+): Autoimmune hemolytic anemia usually d/t ABO/Rh incompatible -DAT (-): Extrinisic: (splenomegaly, heart valve, MAHA [DIC, HUS/TTP, HELLP, KMP], or burns) or Intrinsic: Membrane problems (hereditary spherocytosis or stomatocytosis), enzyme problems (chronic hemolytic anemia, G6PD or PK deficiency), or hemoglobin problems (sickle cell)

Question 29

Macrocytic anemia

Answer 29

- B12/folate deficiency: will see hypersegmented neutrophils as well as RBC; B12 is absorbed in terminal ilieum so ulcerative colitis or ilium transction pts may be deficient; *imp to give B12 right away becuz that can cause neuro problems where folate doesn't affect neuro - bone marrow failure syndrome (aplastic anemia, BDA, SDS, FA): Hgb F will be elevated - hypothyroidism - down syndrome - reticulocytosis

Question 30

Sickle-cell dx

Answer 30

Def: inherited disorder of erythrocytes SS: pain Eval: peripheral blood smear ``` Txt: Hydroxyurea cellular modulation (prevent dehydration) extra-cellular modulation - dilate vessels - reduce platelet adhesion - anticoagulation - increase arginine Oral iron chelation bone marrow transplantation gene therapy ```

Question 31

Hemolytic Anemia SS and Types

Answer 31

``` Pallor Anemia Icterus, jaundice - indirect bilirubin poor feeding splenomegaly dark urine increased retic count peripheral smear ``` ``` Types: Membrane - Hereditary Spherocytosis - Hereditary Elliptocytosis - Infantile Pyropoikilocytosis - Hereditary Stomatocytosis Enzymes - G6PD - Pyruvate Kinase Hemoglobin - Heme synthesis - Globin chain synthesis + thalassemias + hemoglobinopathies ```

Question 32

Alpha Thalassemia summary

Answer 32

Def: absence of 1 - 4 alpha chains in hemoglobin SS: Asymptomatic - missing 1 or 2 splenomegaly/microcytic anemia - missing 3 hydrops fetalis - missing 4 Types: Hb H - 3 gene deletion Hb Barts' - 4 gene deletion Txt: transfusions stem cell transplant

Question 33

Methemoglobinemia summary

Answer 33

Def: normal heme group is Fe3+ and holds onto Oxygen more tightly Cause: Drugs Nitrates (well water) Congenital SS: chocolate brown blood cyanosis MetHb > 10-15% Txt: remove inciting agent oxygen methylene blue (not with G6PD)

Question 34

Neonatal Alloimmune Hemolytic Anemia (HDN) summary

Answer 34

Def: maternal alloantibody directed against fetal antigens either Rh (not first preg), ABO (first preg), or other groups

Question 35

Warm Reactive Autoimmune Hemolytic Anemia summary

Answer 35

Def: IgG mediated antibodies against Rh antigens eval: DAT (coombs) ``` Txt: steroids splenectomy immunosuppressive drugs IVIG ```

Question 36

Cold Agglutinin Dx summary

Answer 36

Def: IgM mediated complex forms at 4 C eval: DAT (coombs) Txt: keep pt warm supportive therapy plasmapheresis Rituximab

Question 37

Paroxysmal Cold Hemoglobinuria summary

Answer 37

Def: acut illness IgG anti-P leading to intravascular hemolysis ``` Cause: measles mumps varicella syphillis mycoplasma ``` txt: supportive care

Question 38

Leukemia

Answer 38

split into acute or chronic and myeloid or lymphoid S/s: fatigue, infection, mild bleeding symptoms; abnormal CBC of pancytopenia or marked leukocytosis Dgx: detailed Hx and PE; labs: CBC, renal and liver function, coags, peripheral blood immunophenotyping; Bone marrow and biopsy; imaging studies Acute: blocks mature B cells from being made; meyloid stem cell side is unaffected - ALL: risk factors (radiation, viral infection, genetic disorders); kids most common and highly curable; adults less common and requires transplant - AML:risk factors (radiation, chemo, benzene, tobacco smoke, genetic disorders, preexisting MDS); uncommon in kids, prognosis depends on type Chronic - CLL-risk factors (white males over 70); not seen in kids; no tx until sx develop; cure is unlikely - CML: risk factors (radiation); very uncommon in kids, up to 70% cure if transplanted; Philedelphia chromosome and Gleevec Tx: not a surgical ds, chemo is main tx

Question 39

Lymphoma

Answer 39

cancer of lymphoid tissues; split into localized or generalized S/s: fatigue, wt loss, fever, night sweats, lymphadenopathy, enlarged liver and/or spleen, skin lesions, pruritis, GI bleeding, obstruction Dgx: detailed Hx and PE, labs: CBC renal and liver function, coags, LDH, peripheral lood immunophenotyping; CT, MRI, PET; biopsy Hodgkin's Disease: relatively uncommon, affects young adults; high cure rates Non-Hodgkin Lymphoma: common, affects older adults; wide variation in prognosis Tx: not a surgical disease, chemo, radiation, stem cell transplant

Question 40

Monoclonal Gammopathy of Undetermined Significance

Answer 40

Common-occurs in those over 70 yo Most pts die of unrelated causes 10-20% of these may progress to myeloma in 10 years (but again most people are dead of something else first)

Question 41

Multiple myeloma

Answer 41

median age: 65 yo incidence in AA is twice of whites S/s: renal failure, hyperviscosity, amyloidosis, bone pain, pathologic fracture, hypercalcemia, anemia, infections Myeloma = 1 major + 1 minor criteria or 3 minor criteria (look in other flashcard section for criteria) Tx: transplants, new drugs work better and have higher response and complete remission rates

Question 42

Myelodysplastic Syndromes

Answer 42

Def: heterogenous group of malignany hematopoietic stem cell disorders; clonal process thought to develop from a single stem cell disorder; characterized by ineffective hematopoiesis and dysplastic cells median age 65 yo risk factors: benzene, radiation, tobacco, chemo, and genetic factors Dgx: ineffective hematopoiesis, dysplastic cells in the blood and bone marrow, cytogenic abnormalities, presence of circulating blasts Tx: observation, supportive care, chemo, transplant

Question 43

Myeloproliferative Neoplasms

Answer 43

like MDS BUT is characterized by excessive proliferation of mature blood cells Specific entities: polycythemia vera, essential thrombocythemia, chronic myeloid leukemia, primary myelofibrosis S/s: fatigue, pruritis, splenomegaly, hypermetabolic sx, bone pain, neuro sx Dgx: elevated blood counts, some found incidentally Tx: no current therapies to prevent this from progressing to AML or marrow fibrosis; PV and ET prevent thrombotic complications; CML TKI therapy; PMF JAK2; transplant is only curative

Question 44

HIV summary

Answer 44

Def: retrovirus that does reverse transcriptase in host cell and convert their RNA into DNA Cause: fluid, blood, tissues from infected person ``` Eval: One of these: Serum antibody Saliva and urine antibody test Rapid tests - SUDS finger stick - OraQuick ``` Confirm with this: Western blot assay ``` SS: Fever Fatigue Rash HA ```