Hematological Malignancies

Lymphoma:

a. Hodgkin’s lymphoma:

  • Sx: constitutional, localized, single group of nodes, contiguous spread
  • Associated w EBV. Bimodal distribution (common in 20’s and 50’s)
  • Histopathology: Reed-Sternberg cells = tumor giant cells “owl eyes” -> CD15+ and CD30 B-cells
  • Dx: excisional biopsy of LNs

Types:

  • Nodular sclerosis: most common
  • Lymphocyte rich: best prognosis
  • Mixed cellularity: eosinophilia, immunocompromised pt
  • Lymphocyte depleted: immunocompromised pt

Tx:

  • Stages 1 + 2 -> radiotherapy (after negative staging laparotomy and BM biopsy)
  • Stages 3 + 4 -> chemotherapy (ABVD): Adria
    mycin
    (cardiomyopathy), bleomycin
  • (pulmonary fibrosis), vincristine (peripheral neuropathy), dacarbazine (myelosuppression)

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b. Non-hodgkin’s lymphoma: 

  • Sx: constitutional, multiple LN\extra-nodal involvement, noncontiguous spread, nerve palsies
  • Associated w HIV and autoimmune diseases
  • Dx: excisional biopsy of LN
  • Histopathology: majority are B-cell, some are T-cell origin
  • Types:
Type Associations Comments
Neoplasms of mature B-cells
Burkitt lymphoma – t(8;14) [c-myc]

– EBV

– “Starry night” appearance

– Jaw lesions in Africa = endemic

Diffuse large B-cell lymphoma Most common type of non-Hodgkin’s lymphoma in adults
Follicular lymphoma t(14;18) Presents w painless “waxing and waning” LAP
Mantle cell lymphoma t(11;14) Very aggressive, usually present w late-stage disease
Marginal zone lymphoma t(11;18) Associated w chronic inflammation (Sjogren’s, chronic gastritisMALT)
Primary CNS lymphoma HIVAIDS, EBV – AIDS-defining illness

– Mass lesion on MRI needs to be distinguished from toxoplasmosis

Neoplasms of mature T-cells
Adult T-cell lymphoma – HTLV

– IV drug abuse

– Cutaneous lesions

– Lytic bone lesions + hypercalcemia

  • Tx: chemo (R-CHOP); Rituximab (sudden death, TLS), cyclophosphamide (hemorrhagic cystitis, cancer), hydroxy-adriamycin (myelosuppression), “Oncovinvincristine (peripheral neuropathy), prednisone

 


Leukemia:

a. Acute leukemias (AML + ALL):

  • Risk factors: chemoradio, myeloproliferativemyelodysplasticsideroblastic, down syndrome
  • ALL = children, AML = adults
  • Acute promyelocytic leukemia (APL) or M3 -> associated w DIC
  • Labs: ↑-↓ WBC, pancytopenia
  • Peripheral smear: blasts

Best initial diagnostic test? BM biopsy (to dx leukemia; you need >20% blasts on biopsy)

  • AML -> Auer rods, myeloperoxidase +ss
  • ALL -> CALLA and TdT +

Tx: chemo; induction then consolidation:

  • APL -> all-trans retinoic acid (vit A)
  • AML -> cytosine arabinoside (AraC) + daunorubicin
  • ALL -> daunorubicin, vincristine, prednisone + intrathecal methotrexate

 

b. Chronic lymphocytic leukemia (CLL):

  • B-cells
  • Labs: lymphocytosis, hemolytic anemia, thrombocytopenia
  • Peripheral smear: smudge cells
  • Richter transformation: CLL -> high-grade lymphoma (most commonly diffuse large B-cell lymphoma)

Staging:

  1. Elevated WBC onlybbb
  2. Lymphadenopathy
  3. Splenomegaly
  4. Anemia
  5. Thrombocytopenia

Tx:

  • Younger healthy pts -> fludarabine + rituximab
  • Older pts or mild cases -> chlorambucil
  • Refractory cases -> cyclophosphamide
  • Autoimmune thrombocytopenia or hemolytic anemia -> prednisone

 

c. Hairy cell leukemia:

  • B-cells
  • Presentation: massive splenomegaly, pancytopenia, dry tap on aspiration (causes marrow fibrosis and hypercellularity)
  • Best initial test? peripheral smear: B-cells w filamentous projections
  • Most accurate test? Flow cytometry immunotyping
  • Tx: pentostatin or cladribine

 

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d. Chronic myelogenous leukemia (CML):

  • Neutrophils (usually)
  • Dx: Philadelphia chromosome = t(9;22) -> BCR-ABL
  • Labs: ↑ WBC (neutrophils)
  • Peripheral smear: ↓ leukocyte alkaline phosphatase (LAP) score

Tx:

  • Best initial tx: Imatinib (tyrosine kinase inhibitor)
  • Most definitive tx: BMT

Complications:

  • Leukostasis reaction: SOB, blurred vision, priapism, ↑↑ WBC. Tx? Leukopharesis
  • Blast crisis: sudden drop in all cell lines. BMB shows ↑ blasts. Tx? BMT or chemo

 


Myelodysplastic syndrome (MDS): 

  • Preleukemia disorder in older pts, most commonly presenting as asymptomatic pancytopenia
  • CBC: anemia, ↑ MCV, nucleated RBCs, some bl555asts (severity is based on percentage of blasts)
  • Bone marrow -> hypercellular
  • Peripheral smear -> Pelger-Heut cells
  • Prussian blue stain -> ringed sideroblasts
  • Tx: transfusion, erythropoietin, Lenalidomide (w 5q deletion)

 


Multiple myeloma: 

  • Abnormal proliferation of plasma cells -> overproduction of useless monoclonal Ig (IgG or IgA)crab

Clinical presentation: “CRAB

  • HyperCalcemia:
  • Renal failure
  • Anemia (normocytic normochromic)
  • Bone lytic lesions Back pain

Investigations:

  • X-ray: punched out lytic lesions
  • Serum protein electrophoresis: M spike
  • ABGs: low anion gap
  • Peripheral smear: Rouleaux formation of RBCs (stacked like poker chips)
  • BMB: >10% plasma cells
  • Urinalysis: Bence Jones proteins (Ig light chains)

Tx: lenalidomide, dexamethasone, autologous BMT

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Polycythemia vera:

  • Overproduction of RBCs due to JAK2 mutation (which regulates marrow production)
  • Sx of hyperviscosity: HTN, splenomegaly, bleeding, thrombosis, pruritus, erythromelalgiappp
  • Labs: ↑ Hct (>60%), ↑ plts and WBC, ↓ erythropoietin, ↓ MCV, ↑ vit B12
  • Tx: phlebotomy, aspirin, hydroxyurea, allopurinol, antihistamines 

 


Download the PDF version: here


References:

  • Kaplan lecture notes
  • Paul Bolin’s vids
  • First aid USMLE step 1
  • Master the boards

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