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H46 Hematology
Lymphomas Toronto Notes 2019
■ cardiac function assessment (MUGA scan or echocardiography): for patients at high risk of pre-treatment cardiac disease (age >60, history of HTN, CHF, PUD, CAD, MI, CVA, and malnourished), treatment can be cardiotoxic
■ PFTs: if history of lung disease (COPD, smoking, and previous radiation to lung)
• excisionallymphnodeorcorebiopsyconfirmsdiagnosis
• bonemarrowbiopsytoassessmarrowinfiltration(onlynecessaryifB-symptoms,stageIIIorIV,and
bulky disease or cytopenia)
Treatment
• stageI-II:chemotherapy(ABVD)followedbyinvolvedfieldorinvolvedsiteradiotherapy(XRT) • stageIII-IV:chemotherapy(ABVD)withXRTforbulkydisease
• relapse,resistanttotherapy:highdosechemotherapyandautologousstemcelltransplant
■ PET scan results essential in clarifying disease response
Complications of Treatment
• cardiacdisease:secondarytoXRT,adriamycinisalsocardiotoxic
• pulmonarydisease:secondarytobleomycin(interstitialpneumonitis) • infertility:recommendspermbanking
• secondarymalignancyinirradiatedfield
■ <2% risk of MDS, AML (secondary to treatment, usually within 8 yr) ■ solid tumours of lung, breast; >8 yr after treatment
■ non-Hodgkinlymphoma
• hypothyroidism:postXRT
Prognosis
• Hasencleveradverseprognosticfactors: 1. serum albumin <40 g/L
2. hemoglobin <105 g/L
3. male
4. stage IV disease
5. age ≥45 yr
6. leukocytosis (WBC >1.5 x 109/L)
7. lymphocytopenia (lymphocytes <0.06 x 109/L or <8% of WBC count or both)
• prognosticscore
■ each additional adverse prognostic factor decreases freedom from progression at 5 yr (FFP)
Non-Hodgkin Lymphoma
Definition
• malignantproliferationoflymphoidcellsofprogenitorormatureB-orT-cells
Classification
• multipleclassificationsystemsexistatpresentandmaybeusedatdifferentcentres • canoriginatefrombothB-(85%)andT-orNK-(15%)cells
■ B-cell NHL: e.g. diffuse large B-cell lymphoma, follicular lymphoma, Burkitt’s lymphoma, and mantle cell lymphoma
■ T-cell NHL: e.g. mycosis fungoides (skin), TCL-NOS, and anaplastic large cell lymphoma • WHO/REALclassificationsystem:3categoriesofNHLsbasedonnaturalhistory
■ indolent (35-40% of NHL): e.g. follicular lymphoma, small lymphocytic lymphoma/CLL, and mantle cell lymphoma
■ aggressive (~50% of NHL): e.g. diffuse large B-cell lymphoma
■ highly aggressive (~5% of NHL): e.g. Burkitt’s lymphoma
Clinical Features
• painlesssuperficiallymphadenopathy,usually>1lymphnoderegion
• usuallypresentsaswidespreaddisease(exceptionisaggressivelymphoma)
• constitutionalsymptomsnotascommonasinHodgkinlymphoma
• cytopenia:anemia±neutropenia±thrombocytopeniacanoccurwhenbonemarrowisinvolved • abdominalsigns
■ hepatosplenomegaly
■ retroperitoneal and mesenteric involvement (second most common site of involvement) • oropharyngealinvolvementin5-10%withsorethroatandobstructiveapnea
• extranodalinvolvement:mostcommonlyGItract;alsotestes,bone,andkidney
• CNSinvolvementin1%(oftenwithHIV)
Investigations
• CBC
■ normocytic normochromic anemia
■ autoimmune hemolytic anemia rare
■ advanced disease: thrombocytopenia, neutropenia, and leukoerythroblastic anemia
• peripheralbloodfilmmayshowlymphomacells
Treatment of HL depends on stage; treatment of NHL depends on histologic subtype
International Prognostic Factors Project 1998
Prognostic Factors FFP
0
1
2 67%
3 60%
4 51%
5-7 42%
FFP = freedom from progression at 5 yr
84% 77%
NHL: Associated Conditions
• Immunodeficiency (e.g. HIV)
• Autoimmune diseases (e.g. SLE) • Infections (e.g. EBV)