Chapter 11 Flashcards
DIC
disseminating intravascular coagulation
what is thrombocytopenia?
reduction in platelets
where does hematopoiesis? Adults? Children?
A. Pelvis, cranium, vertebral bodies, sternum, ribs Kids: Marrow of loong bones, tibia, femur. Extramedullary hematopoiesis—-spleen, liver, lymph noes, thymus
simply put, what will hyperplasia of RBC stem cells cause?
increased erythropoiesis
what is a reticulocyte?
immature RBC—1% of all RBC’s mature after 1 day of circulation
reticulocytopenia……
decreased reticulocytes—- less than 0.5% = marrow failure
prevalence of anemia in U.S.?
4% of men and 8% of women
causes of anemia
blood loss, inc RBC destructionm, dec RBC production, malaria?
anemia causes tissue hypoxia, how does kid respond?
kidneys increase erythropoietin (EPO) increases eythropoiesis up to 8x
presentation of anemia
pallor,m fatigue, weakness/lassitude (cloudy) dec growth, osseous abnorm, cachexia
what can hemolytic anemia do?
Hb–> bilirubin–> jaundice/gallstone
types of anemia cellular color
Microcytic, normocytic, macrocytic hypochromic, normochromic, hyperchromic
hemorrhagic anemia, where do you lose the blood
G.I/ gynecologic pathology. Normocytic/normochromis RBCs
how does body respond to hemorrhagic anemia
inc EPO `3 days inc plasma ~7days inc reticulocytes
Chronic hemorrhagic anemia can cuase IDA
Iron deficiency anemia duh
how much blood loss for hypovolemic shock?
20%
Accelerated RBC destruction
hemolytic anemia
how does body adapt to hemolytic anemia?
Inc. erythropoiesis, reticulocytes, extramedullary hematopoiesis (liver, spleen)
with hemolytic anemia, does IDA appear?
no, the iron is recycled wuite efficiently
Hereditary: abnormal RBC membranes, enzymes deficiency, disordered Hb synthesis
Intracorpuscular defects (inside the RBC) dont for get hereditary!
antibodies, RBC trauma, infxns (malaria) Acquired!
extracorpuscular
Where does hemolysis occur? Inside peripheral circulation (vessels) Trauma : physical or biochemical damaged heart valve, toxins, heat
intravasuclar hemolysis releases hemoglobin inc Hb in urine = hemogloblinuria Inc bilkirubin = jaundice & gallstones
Extravascualr hemolysis happens where? What are we gonna see?
spleen or liver. RBC damage, antibody opsonization splenomegaly, jaundice
abnormal RBC membrane = inc fragility autosomal dominant mutation
Hereditary spherocytosis
Hereditary sphercytosis severity? more about RBCs
anemia MC is moderate decreased elasticity look at more under hereditary spherocytosis—you werent paying attendtion anyway
B-globin mutation, autsomal recessive Chronic hemolytic anemia
Sickle cell anemia sickle hemoglobin (HbS)
HbS who gets it
8% african amercans are heterozygous - Carriers translates to 3.5 mil 1 in 600 are homozygous = sickle cell anemia
sickling occurs where? common at areas of stasis–Bone Marrow
decrease in oxygen, may cause microvascular thrombi
here is a ton of things for sickle cell
thrombosis, fever, malaise, chronic low level pain priapism 4 hr erection, splenomegaly/infarction, gallstones Dec growth, osseous distortion
Sickle cell syndromes: lung infxns ort PE produces pulmonary stasis- red O2- thrombosis
Acute Chest syndrome
more syndromes assoc with sickle cell
stroke, infxn/septicemia, hypoxia induced fatty changes
what can happen to sine in homozygous sickle cell anemia? 10 % of sickle cell anemia
“lincoln log vertebra”… H-shaped vertebra
what ahppens in thalassemia
alpha or B globin genes mutated B is B mutation —chromosome 11 alpha is alpha—- chromosome 16
autosomal recessive, abnomral hemoglobin production microcytic, hypochromic
Thalassemia—beta couldnt fill the blood cell up with stuff
the mutation of thalassemia will cause an excess of the other globin chain, what does this do?
damages RBCs—hemolysis damages erythroblasts 2 alpha, 2 beta—but cant make beta—excessive alpha
the decreased beta globin chain synthesis of beta-thalassemia will cause what
unpaired alpha-globin—hemolysis death of erythroblast—decrease of erythropoiesis
beta thallaseemia minor affects 1 allele
subtle hemolysis—aysmptomatic
beta-thalassemia major —2 alleles
severe hemolysis, severe anemia extramedullary hematopoiesis, splenomegaly —stunted growth —-bone marrow expansion/distortion
beta thalassemia is know as beta thalassemia ‘trait’ affects?
asympt or mild, normal life span, microcytic/microchromic Tx: may not be encessary –monitoring –occasional transfusion monitor serum ferritin—iron chelation
beta thalassemia major Dx:
clinical features—severe anemia, stunted growth, skeletal deform, splenomegaly, poikilocytes
Tx of major?
repeated transfusion and iron chelation —dec anemia symptons & deformities —extends life into 20s…ouch =(
more stuff of Major *hair on end”—calvarium xray “lace-like trabeculation”—deformed hands
eventual iron overlaod—“:hematochromatosis” lethal dilated cardiomyopathy—liver damage, failure
alpha thalassemia, what are we gonna see
typically less severe than B thalassemia abnormal Hb—-HBH slight red o2 capacity ineffective erythropoiesis highly variable, 4 alpha globin genes
G6PD defeciency—x-linked
needed to mkae glutathione–antioxidant protects rbcs against oxidative stress—p
G6PD is asympt until exposed to stress
infections or fava beans (favism)
acute onset of
fatigue,pallor, splenomegaly, back/abdominal pain, hemosiderinuria(dark urine)
risks of G6PD
males. africans 10% of AAs areas of endemic malaria
tx?
depends on severity identify/terminate oxidative stress blood transfusion, partial splenectomy
paroxysmal Nocturnal hemoglobinuria (PNH)
dark urine upon waking
the mutation is a PIGA mutation = deactivates complement inhibitors
RBCs have inc complement fixation acceleration with drop pH= sleeping complement mediated hemolysis at night
features of PNH
chronic low level anemia (MC) inc risk for venous thrombosis
Tx of PNH
antibodies that inhibit the MAC marrow transplant (curative)
folate or Vit B12 deficiency— B9 cobalamin
required for DNA replication megaloblastic anemia
what happens in megaloblastic anemia
inadequate dna rep, then production of megaloblasts macrocytes- macrocytic adn hyperchromic
pancytopenia for megaloblastic anemia—finish you bum
finish
rare, poor diet… increased metabolism –elderly, pregnant,m alcoholism, celiac
folate def. anemia folic acid denatured 10-15 mins after cooking
features of folate def. anemia
insidious, fatigue, weakness, sore tongue —no neurologic dys
Dx. of folate def anemia
macrocytes in peripheral blood, dec., serum folate, normal vit b12
`pernicious anemia
vit b12 def anemia. —DNA synthesis and maintenance of neuronal tissue PNS and spinal cord
whom might get pernicious anemia
strict vegans but MC is chronic malab
what are the chronic malabs that interfere with b12
autoimmune gastritis = dec intrinsic factor post gastrectomy
features of vit b123 anemia
fatigue, pallor, weakness, dyspnea demyelination of PNS& CNS (cord) -numbness, tingling, burning —dec proprioception & ataxia
Dx of vit B12 deficiency
macrocytes dec serum vit b12 and normal folate
tx of vit b12 def
injection vit b12 neurologic recovery is unlikely
anemia of chronic disease—-think about the name
inflamm = dec erythropoiesis MC among hospitalized
features of anemia of chronic disease
dec iron binding capacity inc iron storage in the marrow inc serum ferritin
suppression of myeloid stem cells “bone marrow failure”: hypoceluular, inc fat pancytopenia—rbc wbc platelets
aplastic anemia
greater than 1/2 of aplastic anemia is idiopathic alos what
myelotoxic agents: ADRs, toxins, viral infxn such as EBV, CMV, VZV worse diagnosis
autoimmune portion of aplastic anemia
autoreactive T cells that attack marrow
features of aplastic anemia
reticulocytopenia- weakness pallor dyspnea thrombocytopoenia = dec platelets— petechiae granulocytopenia: infxn, fever, chills splenomegaly not there
Tx of immunosuppressive meds
80 % respond transfusions, marrow transplant best prognosis if less than 40 years and no Hx of transfusions
extensive marrow infiltration
myelophthisic anemia
these inflitrate the bone marrow causing myelophthisic anemia
mets to bone MC granulomatous disease—TB, Bridges-Good syndrome lipid storage disease—Niemann PIck Type C
features of myelophthisic anemia
anemia, thrombocytopenia Dacrocytes—tear shaped RBCs
Tx of myelophthisic anemia
address underlying cond. then maybe a transplant?
Epstein BArr virus
HHV 4
Infectious mono
HHV 4
mono
50% of exposed will become infected
20-50% will shed the virus
Nonspecific response to infxn, red skin
Reactive lymphadenitis
Bartonella henselae
cat scratch fever
bacterial
90% of the time its children
2weeks after scratch, lasts 2-4 months
Irregular stellate necrotizing granulomas
cervical and axillary lymph nodes
go back to stuff before this
go back to stuff you missed. ya big dumb
next topic is lymphoid neoplasms
problems with T cell and B cells
big prob is b cells—class switching
lymphatic mets would go to
liver, spleen, marrow, peripheral circulation
leukemia
lymphoma
involves marrow or blood
involve lymphatic tissues
acute leukemias
onset is sudden and stormy
what other features with marrow suppression
anemia, fatigue is MC feature
neutropenia (fever), thrombocytopenia (bleeding)
marrow expansion (bone pain)
lymphadenopathy,
hepatosplenomegaly
why marrow suppression?
results from transformed lymphoblasts “blasts” on marrow biopsy
Acute lymphoblastic leukemia
acute tumors of lymphoblasts
arrested maturation of B cells or T cells
types of acute lymphoblastic leukemia
Pre-B cell tumors (MC): marow/peripheral blood
Pre-& Cell tumors: thymus
who gets ALL
children, young adults
80% of pediatric leukemias
MC dx at age 4, later with T cell age 15-20
Chronic Lymphocytic Leukemia
B cells, cancer combined with immunosuppression
MC leukemia of adulthood, avg age, 60, males 2x
indolent, initially asymptomatic, gradual prog.
CLL > 4,000 lymphocytes per microL
S(small)LL < 4,000 lymphocytes per microL
CLL what happens
tumor cells displace marrow: pancytopenia
anemia, fatigue, cachexia, hepatosplenomegaly generaized lymphadenopathy
