Epidemiology CBC Results Clinical symptoms Diagnostic tests Treatment Prognosis

Minimal deviation malignancies

Polycythemia vera Most common Hemoglobin >16- Usually incidental CBC, Hydration Increased risk for
myeloproliferative 16.5 g/dL finding. Serum erythropoietin Phlebotomy thrombotic events
neoplasm in Hematocrit >48- May have levels (decreased) (CVD, MI)
adults 49% hyperviscosity Bone marrow exam Hydroxyurea
Increased red cell symptoms NOT recommended Aspirin Not fatal on its own
mass (RCM) Plethora, Rule out secondary Clopidogrel (reduce
erythromelalgia causes of risk of thrombosis) May progress to
erythrocytosis myelofibrosis
(Smoker’s
polycythemia)
Essential More common in Platelet > 450 x If platelet >1000 x CBC, Aspirin, clopidogrel Tendency to progress
thrombocythemia (ET) the aging 109/L 109/L, increased Rule out acute (reduce risk of to myelofibrosis
population risk of bleeding from phase response, iron thrombosis)
consumption of deficiency.
Mutation in JAK2, vWF. Cytoreduction
MPL, CALR Molecular study, (Hydroxyurea)
Increased risk of marrow studies
thrombotic events Anagrelide (inhibits
megakaryocyte
differentiation)
Primary myelofibrosis JAK2 mutation Presence of Weakness, easy PBS (check for If prefibrotic Increased risk for
(PMF) megakaryocytic fatiguability, leukoerythroblastosis (asymptomatic) – thrombosis and
Generally in the proliferation and pruritus, bone pain, – dacrocytes, needs no treatment progression to acute
older population atypia with abdominal fullness, nucleated red cells, leukemia
reticulin and/or weight loss, fever, earlier myeloid If symptomatic
collagen fibrosis night sweats precursors – Transfuse with Due to cytopenia:
metamyelocytes and packed RBC and increased risk for
Splenomegaly myelocytes) “left platelets. infection, bleeding,
(extramedullary shift” poor QoL
hematopoiesis) EPO, steroids,
Bone marrow studies androgens.
shows network of
reticulin fibers Splenectomy is NOT
recommended

Ruxolitinib (JAK2
inhibitor) for
splenomegaly

Myelodysplastic Disease of the (Bone marrow Bone marrow Transfusion to help

syndrome (MDS) elderly failure) aspiration and improve cytopenias
biopsy
Cytopenia
 Can occur after Cytogenetic studies Hematopoietic stem
chemotherapy or (karyotyping or cell transplantation
radiation fluorescence in situ remains the only
hybridization – FISH) curative approach
 Deletion 5q Refractory Treat with Good prognosis, does
syndrome anemia and lenalidomide not transform to acute
thrombocytosis leukemia
Severe deviation malignancies
Acute Myeloid More common in (Bone marrow Symptomatic CBC + PBS Chemotherapy (2 Good prognosis:
Leukemia (AML) adults failure) anemia “Rule of thirds” phases – remission t(15:17), inv(16),
1/3 increased WBC induction phase, and t(8:21), mutant NPM1,
Risk factors: Cytopenia Infections due to 1/3 normal WBC consolidation phase) mutant CEBPA
Trisomy 21 neutropenia, 1/3 decreased WBC
(Down’s bleeding “3+7” regimen Intermediate
syndrome) manifestations Anemia + Anthracycline prognosis:
thrombocytopenia (daunorubicin, Normal karyotype, wild
Lymphadenopathy, doxorubicin, type NPM1
or splenomegaly PBS shows idarubicin) for 3 days
myeloblasts + 7 days of Poor prognosis:
Bone pain due to cytarabine Complex karyotype,
marrow expansion Flow cytometry FLT3-ITD mutant
(CD33, CD13, CD34, Allogeneic stem cell
Can also present CD19 positive) transplant
with solid masses (intermediate risk)
(myeloid sarcoma) Karyotyping + FISH
For acute
promyelocytic
leukemia: All-trans
retinoic acid (ATRA)
 Acute Cells have Auer Treat with ATRA Good prognosis
promyelocytic rods, or are bilobed
leukemia Risk for DIC
 Acute monocytic Hyperleukocytotic Gingival hyperplasia Large cells with Risk for DIC
leukemia folded nucleus
 Acute Common in Bone marrow
megakaryoblastic Down’s syndrome aspirate: “dry tap”
leukemia due to fibrosis
Chronic Myeloid Elderly Leukocytosis, Generally PBS – marked Treat with TKI 85% 10 year survival
Leukemia (CML) population, mild anemia, mild asymptomatic increase in WBC – Imatinib rate
exposure to thrombocytopenia presence of cells in Dasatinib
radiation Indolent phase: different stages of Nilotinib
Leukocytosis, maturation Bosunitib
BCR-ABL splenomegaly, mild Ponatinib
mutation cytopenia PBS looks like bone
(chromosome 22 marrow – cells
and 9) Accelerated phase:
 Recurring present are mature Response to TKI
leukocytosis, (as opposed to AML) monitored with
thrombocytosis, “milestone months”
>20% blasts in PBS Karyotyping/FISH
(blastic crisis) showing BCR-ABL
mutation
(Philadelphia
chromosome)
Acute Lymphoblastic Most common Pancytopenia Easy fatigability, CBC+PBS Intrathecal T-cell lineage has
leukemia (ALL) malignancy in bleeding, fever, Presence of chemotherapy poorer prognosis
childhood infections, lymphoblasts
lymphadenopathies, Treat similar to AML
HTLV, EBV splenomegaly. Bone marrow studies
infections Vincristine
Bone pain, Flow cytometry Doxorubicin
leukostasis Steroids
symptoms Lumbar puncture: Cytarabine
Assess CNS Cyclophosphamide
involvement – ALL Methotrexate
can enter the CNS
If with Philadelphia
chromosome:
Imatinib

CAR-T cells:
1. Get WBC from
patient
2. Engineer to
target cancer
cells
3. Reintroduce
WBC to px
Chronic Lymphocytic Older adults Most are CBC + PBS Low stage disease: Generally non-fatal.
Leukemia (CLL) asymptomatic PBS shows “smudge no need to treat Elderly px, more likely
cells” to die of other
Fatigability, Chemotherapy diseases than CML
recurrent infections Bone marrow
aspiration Supportive treatment Potential to transform
“B symptoms” due to into aggressive high-
Night sweats, Flow cytometry immunocompromised grade lymphoma
weight loss, fever state. (risk for (Richter
Cytogenetics autoimmune transformation)
conditions)
Hodgkin’s lymphoma HIV. EBV Lymphadenopathies Lymph node biopsy Chemotherapy Very high cure rate.
(HL) infection (ABVD – doxorubicin,
bleomycin,
 Nonspecific signs “Reed Sternberg vinblastine, Monitoring for late
and symptoms cells” (Owl’s eye) dacarbazine) complications

Solid mass CT-scan to check for

involvement
(staging)

PET-CT to check
recurrence
Non-Hodgkin In children: Same as HL “CHOP”
lymphoma (NHL) diffuse large B- (Cyclophosphamide,
cell lymphoma doxorubicin,
(DLBCL) and vincristine,
Burkitt lymphoma prednisone), usually
in combination with
In adults: DLBCL Rituximab
and follicular
lymphoma more Radiotherapy
common
PET-CT

Autologous stem cell

transplant
Multiple myeloma Older age group “elderly patient with CBC – normocytic, Autologous stem cell Not curable, goal of
(virtually non- bone pain and normochromic transplantation treatment is to prolong
existent before anemia” anemia time to relapse
age 40) Triplet of bortezomib,
“CRAB” symptoms PBS – rouleaux thalidomide, steroid
(hypercalcemia, formation, may also
renal, anemia, bone yield plasma cells Supportive care –
pain) prevention of
High serum crea and fractures and
Renal failure calcium improvement of bone
status
Neurologic sx Serum TPAG (total (bisphosphonates,
protein, albumin, denosumab)
Susceptibility to globulin) – high
infections globulin fraction Myeloma is NOT
curable
Hyperviscosity sx Bone marrow –
sheets of dysplastic
plasma cells

Protein
electrophoresis
(PAGE) – kappa
 restricted light chains
or lambda restricted
light chains (lambda
light chains produce
more kidney
disease)
Amyloidosis Organ specific Apple green Treat similar to
complaints (renal, birefringence on myeloma.
cardiac) congo red staining
Treat individual organ
Macroglossia, symptoms
racoon eyes accordingly