Blood disorders

ljb20's version from 2016-05-25 23:27

Section 1

Question Answer
Anisocytosiselevated RDW so increase in size or volume of RBC
elevated MCHCmicrocytic hyperchromic
decreased MCHCmicrocytic hypochromic
iron deficiency anemiain addition to normal S/S have risk for infections- decreased serum iron ferritin and saturation. have incraesed transferrin and binding capacity. depressed MCV MCH MCHC. microcytic hypochromic. elevated RDW (anisocytosis) bc form elliptocytes
poikilocytosisabnormally shaped RBC ie elliptocytes (pencil shaped in iron)
Plummer Vinson Syndromechronic decrease in iron. koiloncychia and glossitis, esophageal webs and dysphagia, increased esophageal and hypopharyngeal SCC
Hb H disease 3 of 4 mutated Alpha chains (alpha thal) = body compensate by making Hb out of 4 beta chains
Hb Barts diseaseall 4 alpha mutated. make 4 gamma chains. LETHAL
B thalmajor form (symptomatic) when have 2 defected genes. microcytic hypochromic. high lvls of ferritin iron and saturdation. decrease binding capacity and transferrinn. EXTRAMEDULLARY HEMATOPOEISIS, jaundince, hepatosplenomegaly, frontal bossing, and HAIR ON ENDS. widened body of mnd and change in trabecular pattern
B thal smearelliptocytes, target cells and hypochromic microcytic
unique oral manifestations of B12 deficiency1) Pigmentation 2) periodontal fiber attachment loss and BONE LOSS
difference with folate deficiency and b12 deficiecnB12 has increase in methylmalonic acid and neuro symptoms
Anemia of chronic diseaseincreased IFN TNF or IL block EPO, stop Fe released from macropahges (by increasing hepcidin produciton in liver), promote phago of RBC. IRON RESISTENT. bone marrow stain with prussian blue positive bc iron depositions. have decreased serum iron increased ferritin decreased saturation decreased trasnferrin decreased binding capacity.
sickle cellnormocytic but MCHC is elevated. RBC impede blood flow in times of oxygen stress. leads to widespred hemolysis. bc the cells are adherent it recruit in acute inflammatory cells and platelets. PAIN and abnormal NO metabolism
oral manifestations of sickle cell1) altered trabeculation 2) delayed tooth eruption 3) hypoplastic enamel
tx for sickle cellhydroxyurea. stimuate fetal (2 alpha 2 gamma) Hb
hemolytic anemiaIgG warm lyse in spleen. IgM cold lyse in vasculature and liver
smear difference in warm vs. cold hemolyticwarm- no agglutination. see SPHEROCYTES. cold = Agglutination
2 associations of aplastic anemia1) benzene 2) dyskeratosis congenita

Section 2

Question Answer
risk factors for AMLsmoking, chemo, benzene, aplastic anemia, down syndrome (better outcome) NF1
highest incidence leukemiaAML
highest prevalence leukemiaCLL
CMLRARE IN children. philadelphia 9:22
CMMLOLD MALES. it has over 1000 monocytes iin 1 cubic mm of blood. can progress to AML. risk is prior chemo
ALLmost common pediatric cancer. Cytogenetics have prognostic benefit (some have philadelphia). down syndrome assocaites with POORER OUTCOME. children have better prognosis than adults
CLLMALE ADULTS. most prevalent adult leukemia. usually B cell if T cell worse prognosis. use drug tagets CD20
post transplant lymphoproliferative disorderin pts bc of immunosuppression. EBV infected B cells. can progress to B cell nono hodgkin lymphoma
hogkin lymphomaEBV associated. Armipit Neck Groin 3 most common locations. painless LAP weight loss night sweats and hepatosplenomegaly. Classical (nodular sclerosing variant)= REED STERNBERG CELLS. Nodular Lymphocyte predominant = elderly pts with POPCORN cells
3 things with reed sternbergCMV sialadenitis, Rheumatic fever aschoff bodies, hodgkin lymphome nodaulr sclerosing type
indolent forms of NHLfollicular cell, mycosis fungiodies (cutaneous T cell) and MALT lymphoma
aggressive NHLBurkitts, diffuse large B cell, anaplastic large cell, adult T cell lymphoma
follicular lymphomasmall B cell lymphoma 14:18 => BCL2 over expression. germinal centers. Richter syndrome transform to large cell
2 risk factors for MALT lymphomah pylori in stomach, Sjogren in salivary glands
Burkitts8:14 C myc overexpression. EBV associated in endemic form. high aggressive fast expanding mass. histo= STARRY SKY due to macropahges tingible body
Cutaneous T cell lymphomaMycosis Fungioides. Sezary syndrome when the T cells get in blood. tumor cells have CEREBRIFORM nuclei. they have EPIDERMOTROPISM so can manifest as LP lesions and pautier microabscesses
anaplastic large cell lymphomae2:5. induce ALK1. Cd30 positive. present as non healing ulcer in mouth
Adult T cell leukemiaHTLV tax protein. induce NF kappa B and suppress p53 and p16. tumor induced osteolysis => hypercalcemia and they have 4 leaf clover nuclei
Multiple Myelomamultifocal plasmacytomas. in all instances involve Ig heavy chain TL. CRAB (hyper calcemia, renal insufficiency, anemia, lyticin bone disease). AMYLOIDOSIS. Bence Jones protein excreted in urine (ig light chain) and M spike in serum electrophoresis which is usually IgG heavy chain.
L chain type amyloidosissecreted monoclonal Ig Light chain. can be idiopathic or myeloma related. detected in either serum or urine eletrophoresis manifest with MACROGLOSSIA and skin papules and perioorbital purpura have prolonged PT and PTT bc bind clotting factors.

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