Term
| Hemophilia A is a deficiency in what clotting factor? |
|
Definition
|
|
Term
| Hemophilia B is a deficiency in what clotting factor? |
|
Definition
|
|
Term
| What is another name for Hemophilia B? |
|
Definition
|
|
Term
| occupational exposure to ________ puts a patient at risk for development of leukemia |
|
Definition
|
|
Term
| occupational exposure to _______/______ increases the risk of lymphatic and hematopoietic cancers.. |
|
Definition
|
|
Term
| Hemophilia c is a deficiency in what clotting factor? |
|
Definition
|
|
Term
| What's two other names for hemophilia c |
|
Definition
| Rosenthal syndrome and plasma thromboplastin antecedent deficiency (PTA) |
|
|
Term
| What are some common manifestations of hemophilia a and b |
|
Definition
-joint bleeding
-Cns bleeds
-intramuscular hemorrhage
-GI bleeds
-mucous membrane bleeds |
|
|
Term
| What is the genetic inheritance of hemophilia a and b |
|
Definition
|
|
Term
| What two labs are diagnostic for hemophilia a and b, results? |
|
Definition
aPTT and PT
prolonged aPTT and a normal PT |
|
|
Term
| What is the tx for hemophilia a and b |
|
Definition
A: factor viii replacement and desmospressin can be used
B: factor ix concentrates but desmospressin does not play a role |
|
|
Term
| Which hemophilia is due to a vitamin k dependent factor |
|
Definition
|
|
Term
| What is the inheritance pattern of hemophilia c |
|
Definition
| Autosomal, affects males and females, often seen in Jewish people |
|
|
Term
| Are bleeding manifestations predictable in hemophilia c patients as they are with b and a ? |
|
Definition
| No and they do not correlate with factor xi levels either |
|
|
Term
| In a newly diagnosed hemophilia c patient with no bleeding hx and no family hx (which is NOT uncommon) which two things should be Double checked and ruled out ? |
|
Definition
| Nonspecific inhibitor and lupus anticoagulant |
|
|
Term
|
Definition
| FFP before dental extractions or surgery only |
|
|
Term
| What is the genetic inheritance pattern for sickle cell disease |
|
Definition
|
|
Term
| In sickle cell disease, HbS replaces which normal hb? |
|
Definition
|
|
Term
| Which bones are more commonly affected in kids and which in adults with sickle cell disease? What is the name of the vertebral infraction often seen |
|
Definition
Kids- diaphysis of small tubular bones (dactylitis, hand foot syndrome)
Adults- metaphyses and subchondrium of long bones
"H vertebrae" |
|
|
Term
| What are the 3 cardinal signs of sickle cell disease |
|
Definition
1. Hemolytic anemia
2. Painful vaso-occlusive crisis
3. Multiple organ damage from multiple infarcts |
|
|
Term
| What is an important vitamin supplement for sickle cell disease pt's to take |
|
Definition
| Folate! Bc megaloblastic changes secondary to folate deficiency can complicate their anemia |
|
|
Term
| What is the only drug approved by the FDA for the tx of sickle cell disease |
|
Definition
| Hydroxyurea: MOA increases total and feral hemoglobin and retards gelation and suckling of rbcs |
|
|
Term
| What clotting factor is Von willebrand factor a component of |
|
Definition
|
|
Term
| What are the two functions of vWF |
|
Definition
-helps platelets stick to the site if injury and form a clot
-binds to factor 8 to protect it from rapid clearance from circulation |
|
|
Term
| What are some clinical manifestations of Von willebrands disease |
|
Definition
Mucocutaneous blessing
Epistaxis
Gingival bleeding
Cutaneous brushing
Menorraghia
-in severe disease may see hemarthrosis as in hemophilia a |
|
|
Term
| What tests are used in the dx of Von willebrands disease |
|
Definition
-amount of protein: vWF antigen
-vWF activity-- ristocetin cofactors assay
-aPTT: mildly prolonged in 50% of patients due to low levels of factor 8
-PT should be normal |
|
|
Term
| What is the tx for Von willebrands disease |
|
Definition
-depends on the severity of bleeding
-desmopressin: the tx of choice for most bleeding episodes , if this is it enough can be given vWFA containing concentrate (humatate p) which is also used prophylactic ally for surgery
-FFP transfusion can be used in pt's who are refractory to other tx- but should be avoided due to possibility of viral transmission |
|
|
Term
| What medicine should pt's with Von willebrands disease avoid |
|
Definition
|
|
Term
|
Definition
| An abnormal decrease in the number of platelets in the blood |
|
|
Term
| What is idiopathic thrombocytopenic purpura |
|
Definition
| Thrombocytopenia without toxic exposure or a disease associated with decreased platelet count |
|
|
Term
| ITP in kids is often seen after what two instances |
|
Definition
| 1-4 weeks post viral infection or live virus immunization |
|
|
Term
| What is the pathophysiology behind ITP |
|
Definition
Caused by an antibody (IgG or IgM) that binds to platelet membranes and results in FC receptor mediated splenic destruction of antibody coated platelets
-rarely it is the presenting symptom of an autoimmune disease, like lupus |
|
|
Term
| What will labs show in ITP |
|
Definition
| Decreased platelets but coag studies will be normal |
|
|
Term
| What are the main tx options for ITP |
|
Definition
-IV glucocorticoids
-IV immunoglobulin
-splenectomy if refractory to other tx
-thrombopoietic agents if chronic ITP refractory to other tx |
|
|
Term
| What is the Pentad of TTP |
|
Definition
-microangiopathic hemolytic anemia
Thrombocytopenic purpura
Neurologic abnormalities
Fever
Renal dz |
|
|
Term
| HUS shares many common characteristics with TTP but what are two things that are more common in HUS? |
|
Definition
Renal abnormalities are more severe
More common in kids |
|
|
Term
| What is the basic pathophysiology of TTP |
|
Definition
Bland thrombi in the microvasculature of affected organs, consisting mostly of platelets with little fibrin and red cells
-pt's usually have unusually large multimedia of vWF in their plasma and they lack a plasma protease (ADAMST13) that usually breaks these multimers down |
|
|
Term
| What is more common in TTP than in HUS |
|
Definition
| CNS involvement with mental status changes and focal neurological deficits |
|
|
Term
| What are most cases of TTP associated with |
|
Definition
| Deficiency of ADAMST13 activity due to autoantibodies against its activity |
|
|
Term
| What will always eventually be seen on the peripheral smear of a patient with TTP |
|
Definition
|
|
Term
| What two lab tests utilized in TTP are indirect measures of the degree of hemolysis |
|
Definition
|
|
Term
| What is the therapy if choice for TTP |
|
Definition
| Plasma exchange with fresh frozen plasma |
|
|
Term
| What is an adequate response to FFP exchange in a TTP patient |
|
Definition
Disappearance of neurological signs and symptoms
Platelets greater than 50,000
Decrease in LDH |
|
|
Term
| describe the size and the color of RBCs in anemia of chronic disease? |
|
Definition
|
|
Term
| what are some common morbidities that pt's have who are affected with anemia of chronic disease |
|
Definition
| infection, heart disease, DM, RA, malignancy, CKD, endocrine diseases, liver disease |
|
|
Term
| describe the underlying basic pathophys of anemia of chronic disease? |
|
Definition
| low availability of iron (due to cytokines trapping it in the macrophages), suppressed erythropoiesis d/t CKD or inflammatory cytokines, and relative reduced life span of RBCs |
|
|
Term
| what is the serum transferrin and what will it be in anemia of chronic disease and in iron def anemia? |
|
Definition
-protein responsible for carrying iron around the body, it is increased when erythopoietin is enhanced
-decr in anemia of chronic disease bc there is less erythropoietin
-incr iron def anemia bc the erythropoietin is working hard |
|
|
Term
| what is the serum ferritin level and what would it be in anemia of chronic disease and iron def anemia? |
|
Definition
-indicator of available iron stores in the body
-incr in anemia of chronic disease, bc there is enough iron but the body is unable to use it efficiently
-decr in iron def anemia, bc the basis of the problem is lack of iron stores |
|
|
Term
| what is the tx for anemia of chronic disease? |
|
Definition
-treat underlying cause
-r/o other causes: blood loss, iron def, b12 folate def
-if serious and symptomatic, refer to heme for possible erythropoietin stimulating agents or transfusions |
|
|
Term
|
Definition
| a syndrome of bone marrow failure characterized by pancytopenia and marrow hypoplasia |
|
|
Term
| what are the three main manifestations of aplastic anemia and the s/sx that go along with them? |
|
Definition
-anemia: pallor, tachycardia, fatigue, HAs, dyspnea, palpitations
-neutropenia: frequent/prolonged infections, mouth/pharyngeal ulcerations
-thrombocytopenia: easy bruising, bleeding, petechial rashes |
|
|
Term
| describe aplastic anemia in terms of size and color of RBCs |
|
Definition
normocytic or mildly macrocytic
normochromic |
|
|
Term
| What is the tx of aplastic anemia? |
|
Definition
| hematologic emergency, needs to be referred to a physician who is expert in heme and IC care. |
|
|
Term
| what patient populations are at increased risk of folate def anemia? |
|
Definition
-ALCOHOLICS
-pregnant women
-elderly |
|
|
Term
| describe the size of RBCs in folate def anemia. Why? |
|
Definition
-MACROcytic
-there is inadequate thymidine d/t the def of folate which leads to slower DNA replication. enlarged hematopoietic precursors (megaloblasts) in the bone marrow give rise to enlarged RBCs (macrocytes) |
|
|
Term
| what is the most accurate test to measure a person's folate? |
|
Definition
| -measure the RBC folate. more telling of the persons actual levels as plasma folate can change dramatically from day to day based on diet |
|
|
Term
| Homocysteine levels and methylmalonic acid levels are usually measured to dx and differentiate between B12 def and folate dec anemia. Which anemia will have which results? |
|
Definition
-B12 will have both a increased homocysteine and methylmalonic acid
-Folate will ONLY have increased homocysteine! |
|
|
Term
| Why must you rule out B12 def before treating for folate def anemia? |
|
Definition
| Bc supplementing folate will mask the b12 def, but the neuro symptoms that are only a part of B12 def will continue to worsen if the B12 is not replaced. |
|
|
Term
| Hypersegmented polymorphonuclear cells are characteristic of what anemias? |
|
Definition
| B12 and folate def anemias |
|
|
Term
| give some s/sx of folate def anemia |
|
Definition
-glossitis
-vague GI sx
-weakness, fatigue, difficulty concentrating, irritability, HA
-palpitations, dyspnea |
|
|
Term
| what is the tx for folate def anemia? |
|
Definition
| Folate 1 mg PO per day until resolved |
|
|
Term
| what is a common drug that is a folate antagonist? |
|
Definition
|
|
Term
| what role does B12 play in the absorption of folate? |
|
Definition
| B12 is necessary for folate to be taken up in cells |
|
|
Term
| G6PD def is classified as what type of anemia? |
|
Definition
|
|
Term
| How is G6PD def genetically passed? |
|
Definition
|
|
Term
| describe how G6PD def causes a hemolytic anemia |
|
Definition
| -G6PD is a gene that processes glucose and produces NADPH. NADPH is needed to protect RBCs from the harmful effects of reactive oxygen species. Without the G6PD gene, oxidation of RBCs causes a hemolytic anemia |
|
|
Term
| What are three things a patient with G6PD def should avoid? |
|
Definition
-oxidative drugs (sulfa, ASA)
-infections
-FAVA BEANS |
|
|
Term
| what are signs/sx a patient is undergoing an acute hemolytic episode |
|
Definition
|
|
Term
| what will be seen on a peripheral smear of a pt with G6PD def? |
|
Definition
| Heinz Bodies and Bite cells. This is because heinz bodies are from the precipitates of denatured hgb in the cells. Macrophages prematurely lyse these RBCs and turn them into bite cells. |
|
|
Term
| what is the general tx for G6PD def |
|
Definition
-usually self limiting
-avoid oxidative drugs (SULFAs)
-maintain hydration
-replenish RBCs through transfusion when necessary |
|
|
Term
| what type of bean should be avoided in pt's with G6PD def? |
|
Definition
|
|
Term
| you hear heinz bodies and bite cells, you immediately think…. |
|
Definition
|
|
Term
| what is the most basic pathophys of any hemolytic anemia |
|
Definition
| bone marrow activity cannot compensate for erythrocyte loss |
|
|
Term
| Causes of hemolytic anemia are intrinsic and extrinsic. give ex's of each |
|
Definition
-intrinsic: sickle cell dz, G6PD, hereditary spherocytosis, paroxysmal nocturnal hemoglobinuria
-extrinsic: TTP, HUS, DIC, burns, autoimmune and lymphoproliferative disease, hypersplenism, drug box |
|
|
Term
| give some s/sx of hemolytic anemia |
|
Definition
-sx: fatigue, dyspnea, DARK RED URINE, back pain
-s: lymphadenopathy, splenomegaly, hepatomegaly, jaundice, pallor, tachycardia |
|
|
Term
| What lab values are the HALLMARK of hemolytic anemia |
|
Definition
| elevated reticulocyte count in the presence of a stable or falling Hematocrit |
|
|
Term
| what type of bilirubin will be elevated in a hemolytic anemia? |
|
Definition
| unconjugated (indirect) bilirubin |
|
|
Term
| an anemia that presents with low serum iron, low ferritin, and high TIBC is classically what? |
|
Definition
|
|
Term
| why does iron def cause anemia? |
|
Definition
| iron is needed for erythropoiesis, without it erythropoiesis is diminished and hgb production is inadequate |
|
|
Term
| what do the cells of iron def anemia look like? |
|
Definition
|
|
Term
| what is the most common cause of iron def anemia worldwide? in the US? |
|
Definition
-Malnutrition world wide
-in US blood loss |
|
|
Term
| What are some major causes of blood loss in the US that can cause iron def anemia? |
|
Definition
-heavy menstrual cycle
-GI bleed (check FOBT and refer for scope if necessary)
-ALWAYS ask about chronic NSAID use |
|
|
Term
| what is plummer vinson syndrome? |
|
Definition
| -esophageal webbing, postcricoid dysphagia, and iron def anemia |
|
|
Term
|
Definition
| spoon shaped nails seen in iron def anemia |
|
|
Term
| what is the tx of iron def anemia |
|
Definition
-correct underlying cause
-ferrou sulfate 300 mg BID-TID |
|
|
Term
| what are the side effects of supplementing iron? |
|
Definition
| CONSTIPATION, nausea, dyspepsia |
|
|
Term
| between alpha, beta, and gamma globins, which are present in the fetus and which in adults? |
|
Definition
-alpha present in fetus and adult
-beta present in adult
-gamma present in fetus
*gamma globins disappear and beta take over after birth, while alpha are present the whole time |
|
|
Term
| you hear target cells, you think…. |
|
Definition
|
|
Term
| define the following terms: anisocytosis an poikilocytosis |
|
Definition
-anisocytosis: abnormal variation in the size of RBC
-poikilocytosis: abnormally shaped RBCs |
|
|
Term
| what are some signs and sx that are unique to B12 def? |
|
Definition
| peripheral neuropathy, parasthesias, leg stiffness, ataxia, memory impairment, personality change, depressed mood |
|
|
Term
| what lab test will be elevated in b12 def but not folate |
|
Definition
| both will have elevated homocysteine levels, BUT b12 def will have an elevated methylmalonic acid and folate will NOT have an elevated MMA |
|
|
Term
| what is pernicious anemia? what is the test to dx? |
|
Definition
chronic illness caused by impaired B12 absorption d/t lack of intrinsic factor in gastric acid secretions
-must check for ab's to intrinsic factor, or just check the levels of IF in the gastric acid which will be decr or absent |
|
|
Term
|
Definition
| 100 mcg of cyanocobalamin IM qday x 7 days, then QOD x 7 days, then Q3-4 dys for 2-3 weeks, then Qmonth |
|
|
Term
| what are the GI sx of b12 def anemia? |
|
Definition
anorexia
jaundice
glossitis |
|
|
Term
| what is the basis behind factor V Leiden? |
|
Definition
| It is a hyper coagulability DO. Factor 5 is a clotting factor in the blood and ppl with this condition have a mutation which causes factor 5 to be deactivated more slowly--- leading to a hyper coagulable state |
|
|
Term
| What is the basis of Protein C and Protein S Deficiency? |
|
Definition
| -They exert their anticoagulation effects by inactivating factors 5 and 8. When there is a def these clotting factors are not inactivated and the pt becomes hyper coagulable |
|
|
Term
| Heparin potentiates what in the blood to cause anticoagulation? |
|
Definition
|
|
Term
| Describe the basis of antithrombin III def? |
|
Definition
| inhibits coag cascade by lysing thrombin and factor X a, when there is a def the coat cascade is not inhibited and the pt becomes hyperocagulable |
|
|
Term
| what anticoagulation medication can cause hyper coagulation? |
|
Definition
| HEPARIN! heparin induced thrombocytopenia (HIT) |
|
|
Term
| anticoagulation's of what autoimmune disease are associated with acquired hyper coagulability d/t poorly understood mechanisms? |
|
Definition
|
|
Term
| what is a test to determine the presence of lupus anticoagulants? |
|
Definition
|
|
Term
| what is the most common leukemia in children? peak age of onset? |
|
Definition
acute lymphocytic leukemia (ALL)
4 |
|
|
Term
| What are the presenting s/sx of ALL? |
|
Definition
| rapid onset of recurrent infections, easy bruising, fatigue, generalized weakness, and bleeding into the skin and major internal organs |
|
|
Term
| clinically what can be seen on exam of a pt with ALL? |
|
Definition
| enlarged lymph nodes and possibly splenomegaly |
|
|
Term
| what is the cure rate of ALL? |
|
Definition
| with chemo, almost all go into remission and 70% are cured |
|
|
Term
| in ALL, what is the hallmark finding in the bone marrow? |
|
Definition
| >25% of the bone marrow is lymphoblasts, there is massive infiltration of the bone marrow with immature lymphocytes (lymphoblast) |
|
|
Term
| what is the most prevalent of all the leukemias? |
|
Definition
| acute myelogenous leukemia, AML |
|
|
Term
| What is the hallmark laboratory finding of CLL? |
|
Definition
| isolated lymphocytosis, suspect CLL if lymphocytes in the blood is >5000 |
|
|
Term
| What two things will be seen on a peripheral smear of a pt with CLL? |
|
Definition
-mature, small lymphocytes
-smudge cells (the leukemic lymphocytes are fragile, so when they are smeared onto a glass they are broken and turn into smudge cells) |
|
|
Term
| What may be seen on a CXR of a patient with CLL or ALL? |
|
Definition
| enlarged mediastinal lymph nodes |
|
|
Term
| what is the prognosis of CLL? |
|
Definition
| most survive 7-9 years from the time of dx. This disease seems relatively harmless, but is resistant to cure |
|
|
Term
| what is the most common cell line involved in CLL? |
|
Definition
|
|
Term
| what will be seen on the peripheral smear of a patient with AML? |
|
Definition
|
|
Term
|
Definition
| -neutropenia causing incr infections, thrombocytopenia causing mucosal or cutaneous bleeding/easy bruising, lethargy, anorexia, bone and joint pain |
|
|
Term
| What will be seen on the bone marrow biopsy of a patient with AML? |
|
Definition
|
|
Term
| What is the chromosome associated with CML? how does this form? |
|
Definition
-philadelphia chromosome
-translocation between chromosomes 9 and 22 |
|
|
Term
| What are the three phases of CML? |
|
Definition
| chronic, acclerated, and blast crisis |
|
|
Term
| What is the designer drug created to tx CML? |
|
Definition
|
|
Term
| What is the only proven therapy for CML? |
|
Definition
| allogenic bone marrow transplantation |
|
|
Term
| Give a buzz word/defining characteristic for each of the 4 major leukemias. |
|
Definition
-ALL: MC in kids
-CLL: Smudge cells
-AML: MC leukemia, auer rods
-CML: philadelphia chromosome |
|
|
Term
| What is the difference between non-hodgkins and hodgkins lymphoma in terms of lymph nodes involved? |
|
Definition
-NHL: multiple peripheral LNs involved
-HL: localized to a single axial group |
|
|
Term
| What is the difference between non-hodgkins and hodgkins lymphoma in terms of spread? |
|
Definition
-NHL: noncontiguous spread
-HL: orderly contiguous spread |
|
|
Term
| What is the difference between non-hodgkins and hodgkins lymphoma in terms of mets? |
|
Definition
-NHL: extranodal mets common (brain)
-HL: extranodal mets uncommon |
|
|
Term
| When will you see Reed Sternberg cells on peripheral smear? |
|
Definition
|
|
Term
| what are some s/sx of lymphoma? |
|
Definition
swollen lymph nodes in the neck, axillary, and groin
fatigue, fever, night sweats
weight loss
chest pain, coughing, dyspnea |
|
|
Term
| Most lymphomas have a ___ cell phenotype |
|
Definition
|
|
Term
| What type of hematologic malignancy is characterized by: malignant transformation of a single plasma cell that proliferates in the bone marrow and destroys the surrounding bone? |
|
Definition
| multiple myeloma- a malignancy of plasma cells |
|
|
Term
| What will be seen on serum PRO electrophoresis, in the urine, and on X-ray of a pt with multiple myeloma? |
|
Definition
-Monoclonal spikes on electrophoresis
-bence jones proteins in the urine
-lytic lesions are present on x ray |
|
|
Term
| what is the bacteria associated with contact lens wearers who get corneal abrasions? What is the abx to rx> |
|
Definition
pseudomonas
cipro 0.3% solution otic 2 drops q1-4 hours |
|
|
Term
| what is the referral time for a corneal ulcer? |
|
Definition
| URGENT ophtho referral, they could go blind |
|
|
