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98 Cards in this Set
- Front
- Back
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Anemia -
What is it |
Low Hct and Hb relative to
gender and age |
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Anemia -
History/PE |
weakness
DOE high-output CHF severe - angina and syncope iron-deficient - pica (clay, ice chips) PE- pallor of skin & conjunctiva tachy tachypnea inc. pulse pressure systolic flow murmur jaundice (hemolytic anemia) pos. stool guaiac (GI bleed) |
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Anemia -
Evaluation |
CBC - Hgb, Hct, MCV
folate B12 retic count haptoglobin LDH bilirubin iron ferritin TIBC Coombs' DIC panel - D-dimer fibrinogen fibrin split products occult stool |
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Anemia -
Tx |
Treat underlying cause
iron-def. - oral iron supplements iron-def in elderly male - suspect colorectal cancer B12 def - monthly B12 shots chronic renal dis. - EPO lwr threshold for transfusions in CAD pts (anemia can worsen cardiac ischemia) |
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Anemia -
Types of Microcytic Hypochromic |
Iron deficiency
Anemia of Chronic Disease Lead poisoning Sideroblastic anemia Thalassemias |
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Anemia -
Types of Megaloblastic, Macrocytic |
Megaloblastic -
B12 deficiency folate deficiency Macrocytic - alcohol drugs - anticonvulsants sulfa drugs AZT chemo radiation myelodysplastic syndrome |
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G6PD Deficiency -
What is it |
X-linked recessive
Most affected - Black & Mediterranean men G6PD in HMP shunt => NADPH NADPH needed to keep glutathione reduced to detox free radicals dec. NADPH = poor protection against oxidative damage |
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G6PD Deficiency -
History/PE Triggered by? |
Acute, self-limited h. anemia
fatigue jaundice dark urine hemoglobinuria Triggered by oxidative stress/agents - viral or bact. infections metabolic acidosis fava beans sulfonamides primaquine INH dapsone nitrofurantoin aspirin Most common type of oxidative stress - infections |
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G6PD Deficiency -
Evaluation |
CBC - Hct & retic count
bilirubin haptoglobin Heinz bodies spherocytes G6PD enzyme test - can be false negative during or right after episode G6PD-def. RBC hemolyzed young RBCs have normal enzyme (enzyme activity deficient in older cells) |
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G6PD Deficiency -
Tx |
Sxs usually self-limited
avoid meds that cause transfuse - if severe anemia |
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MAHA -
What is it |
Narrowing of blood vessels
due to fibrin (from inc. coag activity) mechanical damage to RBC and platelets as they squeeze through plat. also caught in fibrin DIC TTP HUS schistocytes burr, helmet & triangle cells |
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HUS -
What is it |
MCC of ARF in kids
gastroenteritis Most cases from E Coli O157:H7 |
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HUS -
Manifestations |
MAHA
thrombocytopenia ARF Renal failure dominates |
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HUS -
Lab Findings |
Anemia
thrombocytopenia |
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HUS -
Tx |
Dialysis for ARF
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TTP -
Causes |
HIV
pregnancy OCP individuals < 50 y/o |
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TTP -
Manifestations |
Fever
thrombocytopenia MAHA transient neuro deficits (thrombi in brain) neuro deficits dominate renal failure (thrombi in kidney) splenomegaly |
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TTP -
Lab Findings |
CBC - thrombocytopenia & MAHA
schistocytes LDH - inc. indirect bilirubin - inc. retic count - high |
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TTP -
Tx |
Large-vol plasmapheresis
corticosteroids ASA - (controversial use) splenectomy |
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DIC -
Causes |
obstetric complications -
(amniotic embolus septic abortion placenta abruptio) sepsis transfusion reaction neoplasia trauma pancreatitis rhabdomyolysis burns snakebites |
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DIC -
Manifestations |
blding from venipuncture site
epistaxis hematemesis digital gangrene hypotension |
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DIC -
Lab Findings |
anemia
thrombocytopenia inc. PT/PTT inc. bleeding time inc. D-dimer inc. fibrin split products dec. fibrinogen |
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DIC -
Tx |
Tx underlying condition
transfuse with platelets and cryoprecipitate |
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Sickle Cell Disease -
What is it (biochemically) What triggers it |
Autosomal recessive
valine for glutamic acid in 6th position of B-globin Triggered by - infection dehydration hypoxia hypoxemia (pneumonia) |
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Sickle Cell Disease -
History/Sx |
Vaso-occlusive crises
splenic crises - splenomegaly => autosplenectomy ~ age 6 risk of encapsulated org. (S. pneumo, H. influenzae, N. meningitides) sequestration crises pain crises aplastic crises - B19 crew haircut on XR aseptic necrosis of fem. head osteomyelitis - Salmonella acute chest syndrome vasa recta - vulnerable extramedullary hematopoiesis reticulocytosis acute dactylitis seizure stroke leg ulcers painful priapism |
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Sickle Cell Disease -
Tx |
Hydration and O2 -
dec. sickling analgesia transfusion - (severe anemia acute chest syndrome with respiratory distress) Abx in acute chest syndrome (often have resp. infection) hydroxyurea 23-valent pneumococc vaccine prophylactic pcn < 5 y/o |
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Beta Thalassemia -
What is it |
point mutation
Mediterranean, Asians, Blacks major = Cooley's Anemia, no beta globin chains minor = dec. beta globin |
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Beta Thalassemia -
History/Features |
ineffective erythropoiesis -
RBCs destroyed in bm splenomegaly hepatomegaly crew haircut bone deformities growth retardation jaundice target cells transfusion => secondary hemochromatosis & CHF |
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Alpha Thalassemia -
What is it |
gene deletions -
1 - silent carrier 2 - trait 3 - HbH disease chronic hemolytic anemia splenomegaly 4 - hydrops fetalis Hb Bart = excess g chains |
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Alpha Thalassemia
Beta Thalassemia - Tx |
transfusions
splenectomy folic acid bm transplant deferoxamine subq - inc. urinary excretion of Fe use to prevent iron overload |
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PCV -
What is it |
Myeloproliferative disease
primary polycythemia Inc. in RBC production WBCs and platelets often inc. > 60 y/o inc. risk of conversion to - AML, CML, myelofibrosis hypercellular bm => bm burned out replaced by fibrosis extramed hematopoiesis myeloid metaplasia with myelofibrosis can => Budd-Chiari MCC of erythrocytosis - secondary polycythemia chronic hypoxia secondary to lung dis. |
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PCV -
History/PE |
Malaise
fever pruritis (esp. after a warm shower) vascular sludging - (headache stroke blurred vision angina MI hepatic vein thrombosis claudication) plethora large retinal veins splenomegaly |
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PCV -
Dx |
O2 sat - normal
Hct > 50% RBC mass inc. EPO undetectable WBC & platelets - norm or inc LAP high ferritin not inc. |
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Secondary Polycythemia -
Features |
Due to hypoxia
Inc. in RBCs ferritin not inc. EPO inc. |
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PCV -
Tx |
. Serial phlebotomy -
dec. Hct and sludging . daily ASA - prevent thrombosis . anagrelide - dec. platelets (inhib maturation of megak) . hydroxyurea - myelosuppress |
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Hyperacute Rejection -
What is it How to prevent |
Pt. has preformed antibodies
thrombi form immed. organ can show signs of ischemia before op finished to prevent - check ABO compatibility give lymphocytotoxic agents |
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Hodgkin's Lymphoma -
What is it |
Malignant
orderly spread from one lymph node group to another contiguous spread extranodal rare Reed-Sternberg cells bi-modal incidence curve - 15-40 y/o and > 60 y/o 50% of cases associated with EBV more common in men except NS good prognosis - more lymphoc Types - NS mixed cellularity lymphocyte-predominant lymphocyte-depleted |
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Hodgkin's Lymphoma -
Hx/PE |
Painless lymphadenopathy
or mediastinal adenopathy B symptoms pruritis fatigue hepatosplenomegaly |
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Hodgkin's Lymphoma -
What are B symptoms What do they mean |
Fever > 38.5 C,
night sweats, 10% weight loss over 6 months systemic Sxs marker for more advanced dis. systemic instead of local involvement neg. prognostic for Hodgkin's reflected in staging A = no systemic Sxs B = presence |
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Acute Rejection -
What is it Tx |
Occurs bet. 5 days & 3 months
liver rejection - inc. GGT, alk phos, bilirub kidney rejection - inc. BUN/Cr confirm with Bx Tx - steroids OKT3 (antilymphocyte ab) |
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Hodgkin's Lymphoma -
Dx |
Bx largest node
then CXR consider bm Bx and LP |
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Hodgkin's Lymphoma -
Tx |
Radiation for localized
chemo for advanced/widepread- ABVD or MOPP ABVD - adriamycin bleomycin vincristine dacarbazine MOPP - mechlorethamine oncovin (vincristine) prednisone procarbazine |
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Chronic Rejection -
What is it Tx |
Irreversible,
gradual loss of function mos. to yrs. after transplant no Tx Bx to r/o late, treatable, acute reaction |
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Non-Hodgkin's Lymphoma -
What is it |
Malignant
involves lymphoreticular & GI extranodal common multiple, peripheral nodes noncontiguous spread incidence inc. with age median age 50 y/o risk factor - immunodeficient Types - lymphoblastic - T lymphocytic - B large - B follicular - B Burkitt's - B |
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Transfusion Reactions -
What are they |
Hemolysis
allergic reactions transfusion-related infection |
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Non-Hodgkin's Lymphoma -
Hx/PE |
Painless adenopathy
B Sxs systemic adenopathy hepatosplenomegaly |
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Non-Hodgkin's Lymphoma -
Dx |
Bx for Dx
CXR whole body CT consider bm Bx and LP then staging |
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Non-Hodgkin's Lymphoma -
Staging |
Based on number of nodes
does disease cross diaphragm any B Sxs |
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Transfusion Reactions -
What is hemolysis from |
Preformed Ab lyse donor RBC
MCC - ABO incompatibility due to clerical error also, mislabeled specimens reactions to Ag not tested |
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Non-Hodgkin's Lymphoma -
Tx |
Radiation and chemo (CHOP)
CHOP - Cytoxan adriamycin Oncovin (vincristine) prednisone Tx may be complicated by tumor lysis syndrome |
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Transfusion Reactions -
Allergic reactions |
IgA deficiency -
anaphylactic reaction during transfusion |
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Non-Hodgkin's Lymphoma -
What is Tumor Lysis Syndrome |
From tumor cell death
releasing intracellular contents after chemo mainly in acute leukemias and non-Hodgkin's hyperK+ hyperuricemia hyperphosphatemia hypoCa2+ ARF |
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Multiple Myeloma -
What is it Risk factors Complications |
Malignancy of plasma cells
arises within bone marrow they invade and destroy adjacent bone tissue > 50 y/o Blacks more than whites Risk factors - radiation chemical exposure MGUS most significant factor - age Dis. usu recurs - poor prog complications - anemia infection neurological diseases renal failure |
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Multiple Myeloma -
Hx/PE |
Back pain
hypercalcemic Sxs - "stones, bones, abdom moans and psych overtones" pathological fractures fatigue frequent infections - esp. H. influ & pneumococcus PE - pallor fever bone tenderness bone deformities lethargy |
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Transfusion Reactions -
Infections |
Rare
viruses - HBV HCV, HIV, HTLV-1, -2, CMV bacteria - risk highest with platelet transfusion |
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Multiple Myeloma -
Dx |
Serum & urine protein
electrophoresis - (monoclonal IgG gammopathy M(onoclonal) spike, M prot.) Bence-Jones proteinuria - IgG light chains CBC - anemia, thrombocytopenia periph smear - rouleaux RBC electrolytes - low-anion gap full-body skeletal survey - punched-out lytic lesions bone scan - neg. bone marrow Bx - > 10% plasma cells confirms UA - detects albumin need specific test for Bence- Jones protein that involves acidification of urine staging based on - degree of anemia elevated calcium XR findings renal dysfunction |
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Multiple Myeloma -
Tx |
Younger pts. - BMT
older - melphalan & prednisone combination chemo - vincristine adriamycin (doxorubicin) dexamethasone use: in prep for BMT or if dis. progresses after melphalan & prednisone hypercalcemia - hydration & loop diuretics then bisphosphonate |
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Transfusion Reactions -
Hx/PE |
Shortly after administered -
hypotension tachypnea tachy fever/chills hemoglobinuria chest pain discomfort |
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Transfusion Reactions -
Tx |
Stop transfusion
retype pts. blood BC DIC labs assess for free Hb in blood and urine IV fluids mannitol |
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Hemophilias -
What are they |
X-linked recessive
coagulopathy Hemophilia A - dec. factor 8 Hemophilia B - dec. factor 9 |
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Hemophilias -
Hx/PE |
Within hrs. of trauma,
bleed into - joints, muscles, GI, brain soft-tissue hemorrhage hemarthroses intramuscular bleeding GI bleeding excessive bleeding from mild trauma, surgical/dental procedures mild deficiency - (25%+ activity) no Sxs or Sxs during surgery or trauma severe deficiency - (< 5-10% activity) spontaneous bleeding |
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Hemophilias -
Dx |
Inc. PTT
Normal - PT, bleeding time, platelets, Ristocetin cofactor assay mixing study - 50/50 mix pt.'s blood with normal blood if PTT corrected - factor deficiency if PTT not corrected - Ab to factor |
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Hemophilias -
Tx |
Give during episodes or
as prophylaxis - factor 8 concentrate (Hemo A) factor 9 concentrate (Hemo B) desmopressin - mild Hemo A before minor surg procedure inc. endog Factor 8 activity |
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von Willebrand's Disease -
What is it |
AD
MC inherited bleeding d/o qual. or quan. defect of vWF aggregation normal adhesion abnormal vWF - prot needed for plat adhesion dec. platelet adhesion dec. factor 8 |
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von Willebrand's Disease -
Hx/PE |
. Easy bruising
. mucosal bleeding - epistaxis oral bleeding GI bleeding menorrhagia . postincisional bleeding |
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von Willebrand's Disease -
Dx |
Normal -
PT, platelet no. PTT - normal or inc. BT - inc., especially after aspirin Ristocetin Cofactor Assay - normal or dec. response |
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von Willebrand's Disease -
Tx |
Desmopressin - if mild
OCP - menorrhagia von Willebrand's factor - now available transfusion - minimized to dec. risk of transmitting viral infections |
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Hypercoagulable States -
What are the causes |
Inherited -
factor V leiden - MC inherited prot C deficiency prot S deficiency antithrombin 3 deficiency homocystinemia fibrinolysis defects Acquired - prolonged bed rest immobilization MI tissue damage DIC hyperlipidemia vasculitis multiple myeloma lupus anticoag nephrotic syndrome smoking cancer warfarin (on initiation) OCPs pregnancy |
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Idiopathic Thrombocytopenic
Purpura (ITP) - What is it |
Autoimmune platelet d/o
kids - postviral, selflimited Ab against viral Ag on platelets adult - chronic IgG against glycoproteins in platelet membrane females affected 2X as males < 50 y/o Evans' syndrome - ITP with autoimmune h. anemia Can be associated with - Hodgkin's NHL CLL HIV SLE RA |
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Idiopathic Thrombocytopenic
Purpura (ITP) - Hx/PE |
afebrile
no splenomegaly petechiae purpura ecchymoses mucosal bleeding - (epistaxis oral bleeding menorrhagia) oral hemorrhagic bullae |
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Idiopathic Thrombocytopenic
Purpura (ITP) - Dx |
CBC
normal - PT, PTT, DIC BT inc. pos.- platelet-assoc. IgG test periph smear - megathrombocyte bm aspiration - normal or inc. megakaryocytes |
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Idiopathic Thrombocytopenic
Purpura (ITP) - Tx |
kids - self-limited
adults - . corticosteroids - may mask leukemia (CLL) bm aspirate 1st . platelets - if severe, uncontrolled bldg . IVIG & anti-RhoD (if Rh+) - life-threatening hemorrh or severe TCP (< 10,000) . splenectomy - if refractory to meds |
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Leukemias -
What are they |
MC cancer in kids
categorized based on cell origin & differentiation major complications end-stage bleeding infection anemia acute - blast min. differentiation chronic - mature cells more differentiated |
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Acute Lymphocytic Leukemia -
What is it |
Lymphoblasts
pre-B or pre-T kids |
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Acute Lymphocytic Leukemia -
Hx/PE |
Hx-
limp refusal to walk bone pain easy bruising fever PE - pallor widespread petechiae/purpura ecchymoses bleeding adenopathy hepatosplenomegaly |
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Acute Lymphocytic Leukemia -
Dx |
Dec. bm elements
inc. LDH inc. uric acid TdT PAS CD10 (CALLA) CXR LP CT Dx based on bm aspirate |
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Acute Lymphocytic Leukemia -
Tx |
Good prognosis with chemo
daunorubicin vincristine prednisone CNS prophylaxis - intrathecal methotrexate |
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Acute Myelogenous Leukemia -
What is it |
Myeloblasts
neutrophils, eosinophils, basophils (neb) 8 different types adults |
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Acute Myelogenous Leukemia -
Hx/PE |
Hx -
fatigue dyspnea (from anemia) fever frequent infections easy bruising leukemia cutis CNS Sxs M3 - DIC M4 - CNS M5 - CNS and gingival hyperplasia PE - lethargy fever bleeding petechiae/purpura hepatosplenomegaly |
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Acute Myelogenous Leukemia -
Dx |
Dec. LAP
Auer rods myeloperoxidase CD33 Sudan stain M3 - t(15;17) |
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Acute Myelogenous Leukemia -
Tx |
Daunorubicin
ara-c transfusions ABx allogeneic/autogeneic BMT retinoic acid - induce remission of promyelocytic form, M3 neutropenic fever - fever if neutrophil count < 500 cells/microL broad-spectrum ABx and antifungals complication - tumor lysis syndrome |
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Chronic Lymphocytic Leukemia -
What is it |
Lymphocytes
T & B elderly (> 65 y/o) slowly progressive many pts. diagnosed by incidental lymphocytosis good short-term survival poor long-term survival |
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Chronic Lymphocytic Leukemia -
Hx/PE |
Lymphadenopathy
fatigue hepatosplenomegaly |
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Chronic Lymphocytic Leukemia -
Dx |
Absolute periph lymphocytosis
inc. lymphocytes in bm malig transformed CD5+ B smudge cells associated with bcl-2 oncogene CD19 stage 0 - lymphocytosis stage 1 - lymphadenopathy stage 2 - splenomegaly stage 3 - anemia stage 4 - TCP stages 1 & 2 - if Sxs, treat stages 3 & 4 - treat |
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Chronic Lymphocytic Leukemia -
Tx |
Supportive
chemo - chlorambucil & prednisone fludarabine once develop Sxs fatigue, lymphadenopathy, anemia or thrombocytopenia splenectomy & steroids - autoimmune HA and TCP |
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Chronic Myelogenous Leukemia -
What is it |
Myeloid cells
often stable for several yrs. then "blast crisis" prior radiation exposure 40-60 y/o |
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Chronic Myelogenous Leukemia -
Hx/PE |
Early stages -
asymp or mild fatigue, fever, malaise later stage - early satiety LUQ fullness - huge splenomeg bleeding blast crisis - final phase rapidly fatal for most fever bone pain weight loss inc. splenomegaly |
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Chronic Myelogenous Leukemia -
Dx |
Myeloid with basophilia
LAP low B12 - often high definitive Dx - Philly chromosome bcr-abl t(9;22) |
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Chronic Myelogenous Leukemia -
Tx |
Poor prognosis
neg Philly chrom - worse prog if have donor - BMT otherwise, imatinib (Gleevac) BMT - often => GVHD |
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MGUS -
What is it |
Monoclonal Gammopathy
of Undetermined Significance overproduction of particular IG (by plasma cells) no systemic manifestations cause is unknown |
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MGUS -
Dx |
Increased monoclonal IG spike
amounts lower than Multiple Myeloma BM < 5% plasma cells (BM Bx > 10% plasma cells confirms Multiple Myeloma) |
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MGUS -
Tx |
Not necessary
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Vitamin K Deficiency -
What does it lead to? What is it from? |
Leads to decreased production
of factors 2, 7, 9 & 10 from - diet deficiency malabsorption ABx that kill bacteria in colon that make it |
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Vitamin K Deficiency -
Sxs |
Bleeding mimics hemophilia
can occur anywhere check for oozing at venipuncture sites |
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Vitamin K Deficiency -
Dx |
Inc. PT & PTT
PT - increases first is more severe confirm - give vitamin K PT & PTT then correct |
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Vitamin K Deficiency -
Tx |
If severe - FFP and vitamin K
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Liver Disease and Bleeding -
What is it |
Factor 8 and VWF are not
made in liver MC site of bleeding - GI decreased platelets from hypersplenism |
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Liver Disease and Bleeding -
Tx |
Acute Tx - FFP
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