Blood Malignancies-II Prof. Dr. Herman Hariman, a Ph.D, SpPK (KH). Prof. Dr. Adikoesoema Aman, SpPK (KH) Dept. of Clinical Pathology, School of Medicine, University of North Sumatra
WHO classification for Lymphoid Disoders Is quite complicated
WHO Classification B-cell neoplasms T- and NK-cell neoplasms Precursor B-cell neoplasm Precursor T-cell neoplasm Precursor B-lymphoblastic leukemia/lymphoma (precursor B-cell acute lymphoblastic leukemia) Precursor T-lymphoblastic lymphoma/leukemia (precursor T- cell acute lymphoblastic leukemia) Mature (peripheral) B-cell neoplasms Mature (peripheral) T-cell neoplasms B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma T-cell prolymphocytic leukemia T-cell granular lymphocytic leukemia B-cell prolymphocytic leukemia Aggressive NK-cell leukemia Lymphoplasmacytic lymphoma Adult T-cell lymphoma/leukemia Splenic marginal zone B-cell lymphoma (with or w/o villous lymphocytes) (human T-cell lymphotropic virus type I positive) Extranodal NK/T-cell lymphoma, nasal type Hairy cell leukemia Enteropathy type T-cell lymphoma Plasma cell myeloma/plasmacytoma Hepatosplenic gammadelta T-cell lymphoma Extranodal marginal zone B-cell lymphoma of mucosa- associated lymphoid tissue type Subcutaneous panniculitis-like T-cell lymphoma Mycosis fungoides/sezary syndrome Nodal marginal zone B-cell lymphoma (with or w/o monocytoid B cells) Anaplastic large cell lymphoma, T/null-cell, primary cutaneous type Follicular lymphoma Peripheral T-cell lymphoma, not otherwise Mantle cell lymphoma Diffuse large B-cell lymphoma characterized Angioimmunoblastic T-cell lymphoma Mediastinal large B-cell lymphoma Anaplastic large cell lymphoma, T/null-cell, Primary effusion lymphoma primary systemic type Burkitt's lymphoma/burkitt's cell leukemia
Lymphoproliferative Disorders Precursor B and T cell acute Lymphoblastic Leukaemia Mature peripheral p e B cell tumour Mature aueperipheral ea T Cell tumour Hodgkin s Lymphoma
A.L.L-L1 usually 75% call, 25% B-ALL
A.L.L-L2 usually T-ALL
A.L.L-L3 Burkitt s stype ALL
Mature (peripheral) B cell tumour Chronic Lymphocytic Leukaemia Atypical CLL Mantle Cell Lymphoma B-Prolymphocytic Leukaemia Marginal Zone Lymphoma Large Cell Transformation of CLL Follicular Lymphoma Non-endemic Burkitt's Lymphoma Diffuse Large B-cell Lymphoma Myeloma, Plasmacytoma and MGUS Hairy Cell Leukaemia
CLL
CLL BIOPSY
Atypical CLL
Mature (peripheral) B cell tumour Chronic Lymphocytic Leukaemia Atypical CLL Mantle Cell Lymphoma B-Prolymphocytic Leukaemia Marginal Zone Lymphoma Large Cell Transformation of CLL Follicular Lymphoma Non-endemic Burkitt's Lymphoma Diffuse Large B-cell Lymphoma Myeloma, Plasmacytoma and MGUS Hairy Cell Leukaemia
Multiple Myeloma More than 15% plasma cells in the bone marrow. Monoclonal immunoglobulin peak on SPEP more than 3 gm/dl. Presence of Bence Jones protein in urine. Decreased levels of normal immunoglobulins.
Diagnosis i of Multiple Myeloma Cell surface markers: CD38, plasma cell antigen, and cell surface immunoglobulins. Monoclonality can be demonstrated by immunoperoxidase staining with kappa and lambda antibodies.
Diagnosis and Staging Workup Bone marrow biopsy and aspirate Serum protein electrophoresis and immunofixation Skeletal survey Plain x-rays are better than bone scan. Lytic lesions do not show up well on bone scan. Quantitative immunoglobulins
Serum Protein Electrophoresis Alb. α 1 α 2 γ Total protein 7.2 α 2 globulin 0.5 albumin 4.5 β globulins li 0.7 α 1 globulin 0.15 γ globulin 1.4 β Normal Spike Alb. α 1 α 2 β γ Total protein 7.9 α 2 globulin 0.6 albumin 3.9 β globulins li 0.7 α 1 globulin 0.19 γ globulin 2.4 Monoclonal
Monoclonal Immunoglobulin Spike on Serum Protein Electrophoresis (SPEP) Multiple myeloma Non-Hodgkin s lymphoma Monoclonal gammopathy of undetermined significance (MGUS). N t li i ll i ifi t l t Not clinically significant unless present in high quantity (over 3 gm/dl).
Durie-Salmon Staging System Stage I mass for Multiple Myeloma Hemoglobin > 10 g/dl Low myeloma cell Normal bone, or solitary plasmacytoma Low immunoglobulin spike (Mcomponent) IgG < 5 g/dl, IgA < 3 g/dl Bence-Jones protein < 4 g/24h
Durie-Salmon Staging System Stage II mass for Multiple Myeloma Intermediate myeloma cell In between Stages I and III Subclassification A: Normal renal function - serum creatinine level < 2.0 mg/dl B: Abnormal renal function - serum creatinine level ³ 2.0 mg/dl
Durie-Salmon Staging System Stage III mass for Multiple Myeloma Hemoglobin < 8.5 g/dl Serum calcium > 12 mg/dl High myeloma cell Multiple lytic bone lesions on x-ray High M-component IgG > 7 g/dl, IgA > 5 g/dl Bence-Jones protein > 12 g/24h
Hodgkin s and Non-Hodgkin s Lymphoma The most important diagnostic tools in Malignant Lymphoma is the HISTOPATHOLOGY (TISSUE BIOPSY)
Hodgkin s and Non-Hodgkin s Lymphoma All patients having been suspected to have Malignant Lymphoma must undergo BMP for both aspiration and biopsy to show the staging (Ann Arbor) Multiple puncture must be done When (+)ve; it is stage IV
Mantle cel lnhl A high power view reveals some heterogeneity of the lymphoid cells. Large immunoblast like cells are noted with the more mature appearing lymphs.
B-Prolymphocytic Leukaemia
Monotonous population of marginal zone lymphocytes with abundant cytoplasm Monotonous population of marginal zone lymphocytes with abundant cytoplasm form indistinct margin with larger germinal center cells having larger vesicular (clear) nuclei.
Follicular NHL
Burkitt NHL
DLBCL
MM >15% PC
MM
Hairy Cell Leukaemia
Mature (peripheral) T-cell Tumours T-Prolymphocytic Leukaemia Large Granular Lymphocytosis Mycosis Fungoides / Sezary Syndrome Lymphomatoid papulosis and CD30 + cutaneous anaplastic lymphoma Adult T-cell Leukaemia/Lymphoma Nodal T-cell Lymphomas
T-Prolymphocytic Leukaemia
Large Granular Lymphocytosis
MYCOSIS FUNGOIDES
Sezary cells may have an irregular nuclear border. Nucleoli are generally not present.
Adult T cell leukemialymphoma Peripheral blood smear showing abnormal lymphocytes with nuclear indentation or lobulation (Wright's stain).htlv1 Japanese and Carribean
HODGKIN LYMPHOMA
HODGKIN LYMPHOMA, LYMPHOCYTE-RICH, HRC CELLS
HD mixed cellularity Eo, Plasma, Lympho
HD Mixed Cellularity, Eos stained, lymph and plasma
HD Lymphocyte Depleted
HD Nodular Sclerosing
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