Heme/Immuno

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Peripheral smear of hereditary spherocytosis

normaocytic, normochromic
spherical RBCs, lack central pallor
reticulocytosis

Peripheral smear of G6PD def

rbc's w/ heinz bodies
reticulocytosis

Sickle cell peripheral smear

normocytic, normochromic
sickled cells (esp w/ sickle prep)
reticulocytosis

peripheral smear of alpha-thal

variable but all hypochromic rbcs
microcytosis
target cells
stippled inclusion bodies (HbH - 3 gene del)
heinz bodies if spleen removed

peripheral smear of beta-thal

anisocytosis (various sizes)
hypochromia, microcytosis
target cells
stippled / fragments of rbcs

megaloblastic anemia peripheral smear (b12 or folate def)

bone marrow findings

macro-ovalocytes
hypersegmented pmns (>5 lobes)

hypercellular, nuclear/cyto dissynchrony

Fe def peripheral smear

microcytic, hypochromia

aplastic anemia peripheral smear

pancytopenia
rbs are normochromic normocytic

causes of aplastic anemia

radiation
benzene
chloramphenicol
akylating agens
antimetabolites
virus (parvo B19, EBV, HIV)
fanconi anemia
acute hep
immune mediated/idopathic

symptoms of aplastic anemia

fatigue, malaise, pallor, purpura, mucosal bleeding, petechiae, infection

DIC smear findings

helmet-shaped cells and schistocytes

what factor is not synthesized in the kidney

VIII - made in endothelum of vessels

Lab findings for thrombocytopenia

decreased platelets
increased bleeding
no change in PT or PTT

labs for hemophelia A or B

no change in platelets
no change in bleeding
no change in PT
increased PTT

labs for von-willebrand's disease

no change in platelets
increased bleeding
no change in PT
increased PTT (due to vWF protection of VIII)

labs for DIC

decreased platelets
increased bleeding
increased PT and PTT -
fibrin split products

labs for Vit K def

no change in platelets
no change in bleeding
increased PT and PTT

labs for Bernard-Souier dx (definition?)

(defective platelet aggreation = defective GP1b)
decreased platelets
increased bleeding
no change in PT or PTT

labs for Glanzmann's thrombasthenia (definition)

(defect in platelet aggregation = defect in GP IIb/IIIa)
normal platelets
increased bleeding
no change in PT or PTT

Reed sternberg cells

giant cell in Hodgkin's
binnulceate or bilobed w/ 2 halves as mirror images ('owl's eyes')
*necessary but no sufficient for dx*

Burkitt mutation

t(8:14) - c-myc activation

follicular lymphoma mutation

t(14;18) - bcl-2 activation

M3 type of AML (responsibe to all-trans retinoic acid) mutation

t(15;17)

Ewing's sarcoma mutation

t(11;22)

Mantle cell lymphoma mutation

t(11;14) - bcl-1 activation

most common adult lymphoma

follicular lymphoma - indolent course
bcl-2 inhibits apoptosis
t(14;18)

auer bodies (rods)

peroxidase + cytoplasmic inclusions in granulocytes and myeloblast (assoc w/ AML) - M3 type. When released (from Rx) - can cause DIC

adult onset, B cells, mature, found in a focal mass
indolent course

smal lymphocytic lymphoma

very similar to CLL - but focal mass present
no mutation known

Adult onset, B cells, angulated grooved cells in a lymphoid center looking mass, nodular pattern
CD10 +, CD5 -

Follicular lymphoma
most common
t(14;18) - which activates bcl-2 (inhibits apoptosis)

Usually older adults, mixed B T cells (B dominate), large cells, aggressive (but up to 50% curable)
*usually pressents w/ large extranodal mass followed by dissemination

diffuse large cell lymphoma
no mutation known

Adult onset, CD5+, Bcells - similar to small lymphocytic lymphoma but arises from outer zone of the follicle

mantle cell lymphoma
t(11;14)
poor ox

Children, immature T cells, mediastinal mass, aggressive course

lymphoblastic lymphoma
commonly presents w/ ALL
no mutation known

sheets of lymphocytes w/ interspersed macs
*jaw or abdomen/pelvis mass

Burkitt's lymphoma
"starry-sky" smear
t(8;14) or (8;22) [gene moves to heavy chain Ig gene - which activates c-myc]

Children < 10, lymphoblasts, TdT+ (marker for pre-T and B cells)

ALL
CD10+ is most common and most sensitive to Rx
can spread to testes and CNS

adolescents to Adults <40yo , myeloblasts on smear

AML
auer rods

Older adults >60 yo, mature B cells, lymphadenopathy, hepatosplenomegaly, few symptoms, indolent course (px 5-7 yrs w/ low symptoms)
*hypogammaglobulinemia = infections

CLL
smudge cells
warm Ab autoimmune hemolytic anemia
similar to small lymphcytic lymphoma

adults 30-60yo, mature cells of the myeloid line, increased PMNs and metamyelocytes

CML
t(9;22) - TK created
may accelerated to AML (blast crisis
*low leukocyte alkaline phosphatase

A left shift (80% bands) and high leukocyte alkaline phosphatase

leukemoid reaction

what cells arise from the myeloid stem cell?

meyloblast, monoblast (histiocytes), megakaryoblast

Birbeck granules

tennis rackets - on EM
histiocytosis X - langerhans cells form teh monocyte line that infiltrate the lung...
young adults
worse w/ smoking

avg age 70 yo, lytic bone lesions = hypercalcemia, bone pain, B-cell proliferation, frequent respiratory infections

MM
M-spike (monoclonal, usually IgA or IgG
monoclonal light chain proteins (bence jones proteins) in urine
rouleaux formation (rbcs)

prominent splenomegaly, anemia, leukopenia, thrombocytopenia
Rartrate-resistant acid phosphatase +

Hairy cell leukemia - bone marrow bx: single rund nucleus w/ cytoplasm w/ fine fibrillary projections
great px - due to rx

serum sickness

type III hypersensitivity dx
fever, urticaria, arthralgias, proteinuria, lymphadenopathy 5-10days post exposure (usually drugs)

Arthus reaction

type III hypersensitivity - subacute Ab-mediated
intraderma injection of antigen induces Ab's - complexes form in skin --> edema, necrosis, activation of complement

common hypersensitivity dx (7)

type 1DM
MS
Guillain-Barre
Hashimoto's thyroiditis
graft-vs-host
PPD
contact dermatitis

hyperacute rejection

w/in minutes
preformed antidonor Ab's w/in the recipient

acute rejection

cell mediated due to cytotoxic T lymph reacting agains foreign MHC's -
weeks after transplant
reversible w/ immunosuppressants (cyclosporine, OKT3)

chronic rejection

Ab-mediated vascular damage (fibrinoid necrosis)
mo to yrs later
irreversible

graft-vs-host
*present

grafted immunocompetent T cells reject 'foreign' cells
present: maculopapular rash, jaundice, hepatosplenomegaly, diarrhea

IL-12 receptor deficiency:

disseminated mycobacterial infections due to decreased Th1 response (IL-12 must be present for undifferentiated Th cells to turn into Th1s)

hyper-IgM syndrome

defect in CD40 ligand - on CD4 Thelper cells leads to inabilit to class switch.
present early w/ sever pyogenic infections

Wiskott-Aldrich syn
*present*

X-linked defect in ability to mount an IgM respons to capsular polysaccharides of bacteria
(increased IgA(, low IgM
WIPE: Wiskott = infections, thrombocytopenic pupura, eczema

Job's syndrome

faliure of INF-gamma production by helper T cells
PMNs fail to respond to chemotactic stimuli
FATED: Facies (coarse), cold staph Abscesses, retained primary Teeth, increased IgE, Dermatologic problems (eczema)

Leukocyte adhesion deficiency

defect in LFA-1 integrin proteins on phagocytes
early: recurrent bacterial infections, absent pus formation, delayed separation of umbilicus

chronic mucocutaneous candidiasis

t-cell dys specifically agains candida.

selective immunoglobin def

def in sp class of Ig's - possible due to a defect in isotype switching
selective IgA def is most common - sinus and lung infections
*milk allergies and diarrhea are common

Ataxia telangiectasia

defect in DNA repair NZ's w/ assoc IgA def.
present: cerebellar ataxia and spider angiomas (telangectasia)

Common variable immunodeficiency

nomal numbers of circulating B cells, decreased plasma cells (defect in B-cell maturation), decreased Ig, can be acquired in 20-30s

Bite cell

G6PD def

teardrop cell

myeloid metaplasia w/ myelofibrosis

acanthocyte

spiny appearance in abetalipoproteinemia

target cells

HALT
Hbc dx
asplenia
liver dx
thalassemia

poikilocytes

nonuniform shapes in TTP/HUS
microvascular damage
DIC

Burr cell

TTP/HUS

basophilic stippling

TAIL
thalassemias
anemia of chronic dx
iron def
lead poisoning

Heinz bodies

(Hb degredation products w/in the cell)
G6PD def
thalassemia - post splenectomy

causes of microcytic, hypochromic anemia

Iron def
thalassemias
lead poisoning
sideroblasatic anemia


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