HEME Flashcards
MC form of acute leukemia in adults
AML
AML basics
ADULT PT
BLASTS
AUER RODS
AML s/s
epistaxis menorrhagia anemia bone/joint pain infections gum hyperplasia splenomegaly
AML dx
CBC = ↓ RBC, ↓ plt, ↓ mature WBC BM = myeloblasts with Auer Rods and 20%+ Blasts
AML tx
combination chemo/BM transplant
CML patho/etio
PHILADELPHIA CHROMOSOME - translocation chromosome 9 and 22
Adults
CML basics
ADULT PT
PHILADELPHIA CHROMOSOME
↑↑WBC
HYPERURICEMIA
CML s/s
Asxs until blastic crisis (acute leukemia)/incidental
↑ WBC –> hypermetabolic state –> fatigue, night sweats, low grade fever
CML dx
CBC = ↑↑WBC
BM = hypercellular left shift myelopoiesis; <5% blasts
Philadelphia Chromosome
CML tx
usually not emergent
chronic phase - TKI - Tyrosine Kinase i (-inibs)/stem cell transplant
Hyperleukocytosis Phase (WBC 100000+) in AML or CML tx
Leukaphoresis (filter out excess WBCs)
Auer Rods
AML
Philadelphia Chromosome
CML
Blasts > 20% + Auer Rods
AML
Blasts < 20% + marked elevated WBC
CML
MC childhood malignancy
ALL
ALL basics
CHILD FEVER BONE PAIN BLEEDING LYMPHADENOPATHY
ALL s/s
pancytopenia –> FEVER, fatigue, lethargy, bone pain
CNS –> Ha, vision, stiff neck, vomit
(METs MC to CNS and testes)
pallor, fatigue, bruising
splenomegaly, hepatomegaly, +/- mediastinal mass
ALL dx
CBC = pancytopenia BM = hypercellular with > 20% blasts
ALL tx
very responsive to combination chemo (hydroxyurea)
remission 90%
relapse –> stem cell transplant
B CELL
CLL
CLL basics
ADULT
ASXS/INCIDENTAL
FATIGUE
SMUDGE CELLS
CLL s/s
MC ASXS/incidental LEUKOCYTOSIS on CBC
fatigue
lymphadenopathy
hepatomegaly, splenomegaly
CLL dx
CBC = ISOLATED LYMPHOCYTOSIS - ↑ WBC with 80% LYMPHOCYTES
smear = incompetent, well-differentiated lymphocytes with SMUDGE CELLS = fragile B cells smudge during slide prep
BM - INFILTRATED WITH LYMPHOCYTES
CLL tx
early/indolent –> observe
sxs/progressive –> chemo
curative –> stem cell transplant
fragile B cells that smudge during slide prep
SMUDGE CELLS IN CLL
smudge cells
CLL
pancytopenia CBC
hypercellular with 20%+ BLASTS BM
ALL
ALL etio
MC childhood malignancy
peak 3-7 yo
↑ risk in kids 5+ with Down Syndrome
Isolated Lymphocytosis
↑WBC with 80% blasts
Smudge Cells
CLL
Proliferation of a SINGLE clone of plasma cells
↓
↑ MONOCLONAL AB (IgG, IgA)
Multiple Myeloma
BREAK
Multiple Myeloma s/s
Bone pain - esp spine and ribs Recurrent infections - d/t leukopenia Elevated Calcium Anemia Kidney Failure
Multiple Myeloma basics
↑ monoclonal Ab IgG, IgA bone pain, HYPERCALCEMIA BENCE JONES pro on urine punched out bone LYTIC LESIONS ROULEAUX RBC formation
Multiple Myeloma dx
BM = plasmacytosis > 10% + 1+…
serum PRO electrophoresis = monoclonal M PRO spike
urine PRO electrophoresis = bence jones pro ; kappa or lambda light chains
x ray = punched out lytic bone lesions
Multiple Myeloma labs
Hypercalcemia Anemia Rouleaux RBC formation ↑ ESR ↑ Cr
Multiple Myeloma tx
HCT - autologous hematopoietic stem cell transplant
Multiple Myeloma prognosis
poor = 2-4 y with tx, mos w/o tx
Auer Rods
AML
Smudge/Smear Cells
CLL
Philadelphia Chromosome
CML
Child with > 30 % blasts
ALL
Philadelphia + tx
PO chemo (hydroxyurea, imatinib)
Monoclonal Ab PRO spike
Multiple Myeloma
Bence Jones PRO
Multiple Myeloma
Rouleaux RBC formation
Multiple Myeloma
Tumor Lysis Syndrome cause, labs, tx
complication 2-3 d post induction tx for AML/CML
hyperuricemia, ↑ K, ↓ Ca, ↑ Phosphate, AKI
tx - IVF + Allopurinol
Tumor Lysis Syndrome tx
IVF + Allopurinol
Highly Curable Lymphoma
Hodgkin
Hodgkin Lymphoma basics
Bimodal Age 20, 50
EBV
Painless Lymphadenopathy (mass in neck)
Reed-Sternberg Cells/Owl Eye appearance
Hodkin Lymphoma patho/etio
B CELL PROLIFERATION with bi/multilobed nucleus “owl eye” (reed sternberg cells)
BIMODAL - peals 20 yo, 50+ yo
associated with EBV
Hodgkin Lymphoma pts MC come to office for …
painless mass in neck (painless lymphadenopathy) that becomes painful with ETOH
Hodgkin s/s
PAINLESS LYMPHADENOPATHY - firm nontender freely mobile ; etoh may induce pain ; hepatosplenomegaly ; esp supraclavicular, cervical, mediastinal
(UPPER BODY LYMPH NODES)
Advanced - SYSTEMIC “B” SXS - cyclical fever, night sweats, weight loss, anemia, pruritus
Pel-Ebstein Fever
Cyclical Fever
in advanced “B” sxs Hodgkin Lymphoma
d/t ↑ cytokines
Hodgkin dx
EXCISIONAL BIOPSY = REED STERNBERG CELL = large cells with bi/multilobar nucleus “OWL-EYE” d/t B CELL PROLIFERATION
CD15+/30+
MEDIASTINAL LYMPHADENOPATHY
UPPER body lymph nodes
with contiguous spread to LOCAL lymph nodes
extranodal is rare
HODGKIN
PERIPHERAL, MULTI nodal involvement
with noncontiguous EXTRANODAL spread
MC to GI, skin
NON HODGKIN
> 50 yo, ↑ risk with immunosuppression (HIV)
not associated with EBV
NON HODGKIN
Associated with EBV
Bimodal
HODGKIN
B Cell: diffuse large B cell
T Cell, natural killer cells
NON HODGKIN cell type
Reed-Sternberg Cells are pathognomonic - B cell proliferation with bilobed or multilobed nucleus “owl eye”
HODGKIN cell type
HIV pt with GI sxs and painless lymphadenopathy
NHL
more rapid onset of symptoms
NHL
SOB, intussusception, bowel obstruction and abdominal mass
NHL
Peripheral lymph nodes most common
NHL
Systemic “B” sxs are rare in …
NHL
Starry Sky appearance
Burkitt Lymphoma (NHL)
Burkitt Lymphoma (NHL) etio, patho, s/s
Very fast growing NHL originating from B cells
MC in male young adults/kids
MC in Africa - specific geographic distribution
Associated with EBV and AIDS
abdominal pain and fullness - sporadic
jaw involvement
histology = starry sky
NHL extranodal sites
MC GI
2 - SKIN
3 - CND
testes, mediastinal
Treat with rituximab, chemotherapy, variable course
NHL
Chemotherapy, radiation therapy – is highly curable compared to Non-Hodgkin Lymphoma
HL
Reed–Sternberg cells confirm the diagnosis.
HL
low-risk stage IA and IIA disease –> XRT
Stage IIIB, IV –> ABVD chemo - adriamycin, bleomycin, vinblastine, dacarbazine
HL
Starry Sky histology
Burkitt Lymphoma NHL
Owl Eyes
HL
Rouleaux RBC formation
Multiple Myeloma
Auer Rods
AML
Smudge/Smear Cells
CLL (B Cell)
Philadelphia Chromosome
CML
Pancytopenia –> anemia, thrombocytopenia, neutropenia, splenomegaly, gingival hyperplasia, bone pain
AML
Monoclonal (M) PRO spike MC IgG
Multiple Myeloma
class of acquired clonal blood disorders
d/t ineffective hematopoieisis
with apoptosis of myeloid precursors
myelodysplastic syndromes (MDS)
group of diverse bone marrow disorders in which the bone marrow does not produce enough healthy blood cells
MDS
“bone marrow failure disorder”
MDS
MDS etio, causes, prognosis
MC ELDERLY
usually idiopathic
radiation exposure, immunosupps, toxins
variable prognosis but generally poor/progress to acute leukemia
MDS s/s
early - ASXS
Pancytopenia incidental finding
manifestations of anemia, thrombocytopenia, neutropenia
? are a hallmark feature of MDS and are responsible for some of the symptoms that MDS patients experience — infection, anemia, spontaneous bleeding, or easy bruising.
Low blood cell counts
Myelodysplastic syndrome may present as excessive bleeding due to …
thrombocytopenia
In MDS, peripheral blood smear shows at least ? % of immature blood cells.?
10%+ immature blood cells
MDS dx
BM biopsy = DYSPLASTIC MARROW CELLS with blasts or ringed sideroblasts
CBC with peripheral smear = nl/↑ MCV, ↓ reticulocyte, ↓ RBC, ↓ WBC, ↓ plt ; dysplastic RBC, dysplastic WBC, normal plt
Myelodysplastic syndrome are pre-leukemias with a high risk of conversion to ?
AML
MDS tx mainly supportive - mainstay ?
RBC and plt transfusions
Erythropoietin may help reduce # of transfusions
BM transplant only potential cure
low blood cell counts in elderly
MDS
Patients with multiple myeloma are prone to infections with encapsulated organisms such as …
Strept pneumoniae and H influenzae
Igs coat RBC and neutralize the ionic charge than normally repels RBCs
RBC rouleaux formation in Multiple Myeloma
Urinalysis: Ig light chains (Bence Jones protein).
Multiple Myeloma
B symptoms are common - fever, weight loss, night sweats
HL
Peripheral, multiple lymph nodes: axillary, abdominal, pelvic inguinal, femoral.
Non-Contiguous, extranodal spread: GI and skin most common
NHL
Associated with EBV (40% of patients)
HL
Painless lymphadenopathy + Reed-Sternberg cells + Bimodal age distribution (15-35) and (>60)
HL
Strikingly Increased WBC count > 100,000
+ hyperuricemia + Adult patient
CML