final Flashcards
B lymphocytes are derived from
BM stem cells
B cells mature into
plasma cells that secrete IGs
MC IG?
IgG
IgA is in the gut
these IGs are seen in hypersensitivity rxns
IgD/IgE
two heavy and light chains
what is complement
-Lysis of bacteria or blood cells
-opsonize (coat) bacteria/blood cells for phagocytosis
-Immunoglobulins presenting to T-cells
Develops in the spleen, fully developed by 5-6 years of age (we don’t want to remove spleen before this)
AIDS
- RNA virus with 3 groups
1) group specific Ag
2) reverse transcriptase and integrase
3) envelope codes to envelope proteins
when an infected HIV cell goes into replicative phase what is happening?
the provirus will produce mRNA and RNA to create new HIV virions
normal CD4+ count vs AIDS count
normal-800-1000
AIDS-<400
which cells does HIV infect?
CD4 (helper T cells)
how to dx AIDS?
ELISA
Western Blot
may nto be positive for 3 months after exposure
how to treat AIDS?
HAART (higly active anti-retro viral therapy)
type reverse transcriptase inhibitors and a protease inhibitor
Diploid Haploid Aneuploid Hyperdiploid Hypodiploid Psuedodiploid
Diploid = 46 chromosomes Haploid = 23 chromosomes Aneuploid = more or less than 46 chromos. Hyperdiploid = more than 46 chromosomes Hyplodiploid = less than 46 chromosomes Pseudodiploid = 46 with chr rearrangement
tumor suppressor genes inhibit cell division. Loss of one allele may predispose to certain tumors suh as
retinoblastoma
Philadelphia Chromosome + in ALL indicates
hyperdiploidy in ALL indicates
poor prognosis
favorable prognosis
Blast cells fail to differentiate but divide - result is bone marrow failure
acute leukemia
MC form of leukemia in children (3-5)
acute lymphoblastic leukemia
-CD 10+ precursor B type is a marker on the cell that shows equal sex distribution
-CD 10- T type is MC in males
If kid keeps get sick every few weeks, then after a while they have swollen lymph nodes/spleen/liver
Blood count smear will be full of blasts, then do a bone marrow (normal person would have 5% blast cells,25-40%)
Liver/spleen can start to make red cells which causes them to get bigger
AML is seen in
- any age group
- less common in kids than ALL
- Auer rods seen
M0-M7 classification is used for
AML
FAB classification is used for
ALL
mediastinal lymph nodes are common with ALL
Breaking tumor cells down releasing all intracellular contents into blood
tumor lysis syndrome, give allopurinol
when to perform BMT on ALL vs AML
ALL- second remission
AML-first remission
CML is seen in
- middle aged pts
- Ph +
- 70% change to ALL/AML (when this happens they are dead within a year)
when do CML pts need a transplant by?
age 55
CLL is mainly seen in
adults
Mikulicz’s Syndrome = salivary & lacrimal inability to produce these fluids
doesn’t transform into acute
Reed Sternberg cells are seen in
Hodkins disease
how does Hodkin’s present?
Present with painless, non-tender, asymmetrical enlarged superficial nodes, fever, weight loss, night sweats
this histological finding shows the best prognosis for Hodgkin’s
lymphocyte predominance
stages of Hodkins
1-one set of lymph nodes
2-2 sets of lymph nodes on same side of body
3-lymph nodes above and below diaphragm
4-outside involvement
B indicates fever, night sweats, weight loss (10%) - over 6 mos
S indicates splenic involvment
hwo to dx hodgkins
- CXR
- Bone Marrow
- CT
hodgkin’s treatment?
stage I and IIa get radiation
stage IIb, III, IV get radiation and chemo
NHL is a disease of which cells?
B lymphocytes
presentation of NDL
Anemia, neutropenia (infections), thrombocytopenia (purpura)
Hepatosplenomegaly (organ involvement)
Enlarged mesenteric/retroperitoneal nodes
NHL prognosis is based on
histology
HDL tx?
autologous marrow transplant to cure patient if radiation or chemo doesn’t work
Burkitt’s
B-lymphoblastic lymphoma
Massive jaw lesions, extranodal abdominal & ovarian involvement in African children
“Starry Sky”
Multiple Myeloma
-Proliferation of BM plasma cells
-Lytic bone lesions (holes in the bone)
-Bence Jones protein/paraprotein
ex-person walking and falls, has a fracture
clinical features of MM
- bone pain
- anemia
- repeated infections
how to dx MM?
- alk phos
- High BUN (higher the BUN, less life expectancy)
- renal failure
how to tx MM?
- chemo
- allopurinol
- BMT for younger
polycthemia vera
- increased RBCs
- Becomes sludgy/sticky and plugs up vessels in brain causing HAs, blurry vision, dyspnea
secondary polycythemia
-compensatory increase in erythropoieten from
-higher altitudes
-cardiac disease
pulmonary disease
Kid w/rash on butt down to feet
Vessels break leading to bruises
The kid is fine, make sure to test urine and guaiac for bleeding
Treat with steroids, usually self limiting
Henoch-Schoenlein Syndrome
Immune complex hypersensitivity reaction
Usually children following an acute infection
hemolytic uremic syndrome is caused by
- E. Coli
- pt presents with bloody diarrhea
- treat with plasmapharesis and plasma transfusion
ITP
Idk why but you have no platelets
Typically follows infection, abs are continued to be made and they attack platelets
Occurs in the spleen, can remove the spleen and this will cure, increases platelet count a SHIT ton
Rash doesn’t blanch
No treatment necessary just follow the platelets, can give steroids and taper, above 30/40k then leave it alone
IV IG in the hospital will cure
Rh+ give rhogam
intrinsic pathway activates factor
XII—>XI—>IX—->vWF + VIII—–>X
extrinsic pathway activates factor
VII—–>X
crush injury will activate this
factor X becomes….
prothrombin which turns into thrombin
fibrinogen which turns into fibrin
fibrin degradation product is called
d-dimer
hemophilia a
missing factor VIII
how to treat hemophilia A
Give the recombinant medication every day via IV for a normal life
hemophilia b -christmas disease
missing factor IX
how to treat hemophilia b?
This is blood bank stuff, no manmade med
Given every second or third day
VWD
- autosomal dominant
- MC general hemophilia
- helps protect factor XIII
- Platelets don’t function properly, don’t close up the hole
From sepsis, meningitis, etc
Clotting everywhere! Put person on heparin
DIC
DIC triad
- thrombocytopenia, decreased fibrinogen, increased split products (FDP)
