Hematomato

board review

By KT/V
 CLL
• Absolute lymphocyte > 5000
• Monoclonal B cell: CD5, CD 19, 20, CD 23
 Blood smear
• Smudge cells
• Prolymphocyte <10%
– If prolymph>55%... PLL
• Anemia, thrombocytopenia
• AIHA
• ITP
• PRCA
 Prognostic factors
• Ig variable region heavy chain genen mutation
• ZAP 70
• Cytogenetics
• CD 38
• Lymphocyte doubling time

• LDH…. Normal
• May transform to aggressive large B cell/ HL
– Richter’s transformation
 When to treat?
• Constitutional symptoms due to CLL
• Symptom or >10 cm lymphadenopathy
• Symptom >6 cm below LCM, splenomegaly\
• Progressive marrow failure
• Lymphocyte doubling time <6 mo
• Autoimmune cytopenia poorly response to
steroid
 How to treat?
• Alkylating- COP
• Fludarabine
• Rituximab
– First infusion: systemic cytokine release syndrome
– Hypotension, bronchospasm
• Alemtuzumab
– anti CD 52 for fludarabine refractory CLL
 CMMoL
• Age 70 years male
• Myelodysplastic
• Fatigue, wt loss, night sweats

CRITERIA DX
• Monocyte>1000, > 3mo
• Absence of philadelphia chromosome
• Blast <20%
• Dysplasia
• Clonal cytogenetic
– Trisomy 8, deletion 7q
– Translocations 5q31-35; PDGFR, eosinophilia
 Treatment
• All experimental
• Cytopenia– growth factors
• Preleukemic– low dose CMT
• Proliferative– hydroxyurea
• PDGFR translocation– Imatinib
 NHL
• Ann arbor staging
 Indolent
• Grade I, II follicular lymphoma
• B-CLL/SLL
• Marginal zone lymphoma
• Lymphoplasmacytoid lymphoma
• Mycosis fungoides
 Treatment- indolent
• CLL--- Fludarabine, Rituximab
• LPL/Waldenstrom
– Fludarabine, rituximab
– COP
• Marginal zone
– Splenic marginal zone– splnectomy, tx HCV
– Asso. H. pylori– eradicate
• Mycosis fungoides– cutaneous T cell
– Topical treatment, oral isptretinoin
– Sezary syndrome: late occur of nodal and leukemic dz,
poor prog
 Aggressive
• Mantle cell: Male, 70 yr
• DLBCL: CD19, 20
• Primary effusion lymphoma: HHV 8

• Treatment
R-CHOP: Rituximab, Cyclophos, Doxorubicin,
Vincristine, Prednisolone
 Highly aggressive
• Precursor lymphoblastic lymphoma/leukemia
• Burkitt lymphoma
– Tumor lysis syndrome
– t8;14, t8;22
Treatment: CODOX-M
cyclophosphamide, vincristine,
doxorubicin, methotrexate
 Salvage therapy
• ESHAP
– Etoposide, methylprednisolone, cytarabine,
cisplatin
• ICE
– Ifosfamide, carboplatin, etoposide
 HIV-related brain lymphoma
• CD4< 50
• Standard- radiotherapy
• HAART … as soon as possible
 Aggressive T cell
• Adult T cell leukemia: HTLV-1
– Skin rash, LN,L+S+, Ca!, LDH!
Tx: Doxorubicin based
• Angioimmunoblastic lymphadenopathy with
dysproteinemia (AILD)
– Middle age, elderly
– Pruritic skin
– Polyclonal hypergammaglobulin
– Hemolytic anemia
Tx: steroids, CMT
 Multiple myeloma
• Monoclonal: G>L>A>D
• DDX M protein plasma cell symptom
– MGUS: <3 g/dl <10% -
– SMM: >3 >10 -
– Nonsecrete: <3 >10 +
Initial evaluation
Staging
No
maintenance
therapy
 Drugs
• IV bisphosphanate: all patients
– Tx hypercalcemia
– Osteoporosis
– Skeletal complications
– Improve QoL
• Bortezomib: proteosome inh
• Thalidomide: s/e DVT, neuropathy
 Solitary Plasmacytoma
• BM- negative
• TX local radiation
 MDS
• 65-70 yr
• Blood smear: macrocytosis, hypogranular
neutrophils, pseudo Pelger-Huet
• BM: dysplastic, ringed sideroblast, micromega-
hypolobated
• Cytogenetics: 5q-, -7,
trisomy 8
 Treatment
1. Performance status
Poor- transfusion, supportive care
2. Risk
Low/inter: Epo, lenalidomide..5q-, azacytidine
Inter/high: young HSCT
azacytidine, combination CMT
Hypoplastic MDS: CsA, ATG
 PRCA
• Thymoma, CNT, MG, CLL, LGL, 5q-
• Parvovirus B 19
• Erythropoietin, chemotherapy
• HIV

• BM: giant pronormoblasts parvovirus

uninuclear micromega 5q-
 Treatment
• Steroid- moderate dose
• Immunosupress- CsA, ATG
• Rituximab
• Thymoma surgery
• Parvovirus IVIg
 Polycythemia vera
• Hypertension
• Thrombosis
• Pruritus– post bathing
• Erythromelalgia
• Joint pain
• Visual disturbance
 Bone marrow
• BM: hypercellularity, trilineage hyperplasia,
megakaryocyte hyperplasia, dec iron
 Diagnostic criteria, 2008 WHO
• Male > 18.5, female >16.5
• JAK2 V617F
• Subnormal EPO
• BM trilineage
 Treatment
• All patients
– Phlebotomy: Hct male<45%, female <42%
– Low dose ASA
• Inter/high risk: age>60, prior VTE, plt> 1.5 mil
– hydroxyurea
 Essential thrombocytosis
• Platelet> 450,000