Leukemia

Lymph Nodes
Normal 
Abnormal
Soft
 FIRM
Mobile
 IMMOBILE
<2cm
 >2cm
No systemic Sx
 SYSTEMIC SX (Fever, Night Sweats, Weight loss)
 AML
Features
·       MCC adult acute leukemia
·       Median age 65 👴🏻👵🏼
S/S
·       Fatigue is common (other B symptoms unusual)
·       Sx of Cytopenias:
·       Fatigue, weakness (anemia)
·       Bleeding, bruising (thrombocytopenia)
·       Infection (granulocytopenia)
·       No HSM/LN
·       DIC (if APML)
Labs
Cytopenias (leukocytes may be ⤴️, normal or ⤵️)
⤴️ LDH
Blood Smear: Myeloblasts with Auer rods
Dx
 BM Bx: usually HYPERCELLULAR with myeloid blasts
 ALL
Features
Most common childhood cancer
Peak age: 2-5 years
Male > female
S/S
Nonspecific systemic symptoms
Bone pain
Lymphadenopathy
Hepatosplenomegaly
Pallor (from anemia)
Petechiae (from thrombocytopenia)
Can presents w/ PANCYTOPENIA
Dx
BM BX with >25% lymphoblasts
Tx
 Chemotherapy
 CLL
Features
Happens in elderly 👵🏼👴🏻
S/S
·       LN (cervical, supraclavicular, axillary)
·       HSM
·       Mild ⤵️  PLT + Anemia
·       Rx Infections (PNA)
·       Often Asx
Dx
·       CBC
·       ⤴️ WBC (>20,00) with > 80% lymphocytes
·       ⤵️ RBCs 
·       ⤵️ PLTs 
·       ⤵️ IgG in half of patients
·       Smear: Smudge Cells
·       Most accurate test: flow cytometry (CD5, 19, 20, 23)  
Tx
·       If Asx → Nothing ø
·       If Sx / Rapid progression → fludarabine
·       Curative Tx → Stem Cell Transplant
Prgonosis
Median survival: 10 years
Worse prognosis w/:
·       Multiple chain LN
·       HSM
·       Anemia & thrombocytopenia
 CML
#
·       Happens in adults (50 yo)
·       👨🏻 > 👩🏻
Path
·       Philadelphia chromosome translocation t(9;22) 
·       BCR-ABL fusion gene 
·       Tyrosine kinase hyperactivity
S/S
Chronic:
·       B-Sx: fatigue / night sweats / weight loss
·       Fever
·       splenomegaly / early satiety
·       Itchiness after hot baths
Accelerated or blast phase
·       bony pain
·       lymphadenopathy
·       variable skin findings
·       extramedullary mass
·       rapidly enlarging spleen
Dx
CBC
·       ⤴️ WBC ( Nø / Basophils)
·       ⤵️ leukocyte alkaline phosphatase
BM Bx
·       ⤴️ Cellularity
·       immature granulocytes
Most accurate → FISH showing Philadelphia chromosome translocation
Tx
·       Tyrosine kinase inhibitors (Imatinib)
·       Curative → HSCT
Leukomoid Reaction
CML
WBC #
 High (50K)
REALLY HIGH (+100k)
?
 Infection
BCR-ABL Fusion
LAP
 ⤴️
⤵️
 Mature Nø (Metamyelocytes)
Immature Nø (Myelocytes)
Basophils?
 Hairy Cell Leukemia
#
·       Clonal B-cell neoplasm
·       Middle-age/older adults
·       BRAF mutation (Proto-oncogene)
S/S
Pancytopenia due to BM fibrosis
·       ⤵️ WBC (infections)
·       Anemia (fatigue, weakness)
·       ⤵️ PLT (bleeding, bruising)
Splenomegaly (early satiety)
Dx
·       Smear: “hairy” leukocyte
·       BM Bx w/ Flow Cytometry
Tx
Chemotherapy
 Langerhans cell histiocytosis
S/S
·       Lytic bone lesions (Skull, jaw, femur)
·       Skin lesions (purplish papules, eczematous rash)
·       LN. HSM
·       Pulmonary cysts/nodules
·       Central Diabetes Insipidus
Dx
 Langerhans cells on bone/skin biopsy
Tx
·       Chemotherapy (prednisone ± vinblastine)
·       Desmopressin for diabetes insipidus
 Multiple myeloma
Path
 Plasma cell neoplasm produces monoclonal paraprotein (immunoglobulin)
S/S
·       Bone pain, Fx
·       B Sx (weight loss, fatigue, Night Sweats)
·       Rx infections
Dx
·       Normocytic anemia
·       Renal insufficiency
·       Hypercalcemia (constipation, muscle weakness, depression)
·       Monoclonal paraproteinemia (M-spike)
X-ray
 Osteolytic lesions/osteopenia (osteoclast activation)
Multiple Myeloma
Waldenström macroglobulinemia
Clx
·        ⤴️ Ca
·       Anemia
·       Renal insufficiency
·       Osteolytic lesions
·       Hyperviscosity (clotting)
·       Bleeding
·       HSM / LN
Monoclonal antibody
 IgG, IgA, light chains
IgM
Smear
Rouleaux
Rouleaux

Leave a comment