Benign Qualitative White Blood Cell (WBC) Disorders
Pathogenesis 1. Defects in leukocyte structure o Example-membrane fusion defect in Chdiak-Higashi syndrome . Defects in leukocyte function a. !eukocyte adhesion defect Example-deficient selectin or CD11a"CD1# b. Phagocytosis defect Example-decreased opsonins in $ruton%s agammaglobulinemia c. &icrobicidal defect Example-deficiency of myeloperoxidase Benign Qualitative White Blood Cell (WBC) Disorders Pathogenesis 1. Defects in leukocyte structure o Example-membrane fusion defect in Chdiak-Higashi syndrome . Defects in leukocyte function a. !eukocyte adhesion defect Example-deficient selectin or CD11a"CD1# b. Phagocytosis defect Example-decreased opsonins in $ruton%s agammaglobulinemia c. &icrobicidal defect Example-deficiency of myeloperoxidase Pathogenesis 1. Defects in leukocyte structure o Example-membrane fusion defect in Chdiak-Higashi syndrome . Defects in leukocyte function a. !eukocyte adhesion defect Example-deficient selectin or CD11a"CD1# b. Phagocytosis defect Example-decreased opsonins in $ruton%s agammaglobulinemia c. &icrobicidal defect Example-deficiency of myeloperoxidase Clinical findings 1. 'nusual pathogens (e.g.) coagulase-negati*e Staphylococcus+ . ,re-uent infections and gro.th failure in children /. !ack of an inflammatory response (e.g.) production of 0cold0 abscesses+ 1. 2e*ere gingi*itis 3ob%s syndrome is an autosomal recessi*e disorder of neutrophils) characteri4ed by abnormal chemotaxis leading to 0cold0 soft tissue abscesses due to Staphylococcus aureus. Patients ha*e red hair) a leonine face) chronic ec4ema) and increased 5gE (hyperimmune E syndrome+. 'nusual benign leukocyte reactions 1. !eukemoid reaction a. 6bsolute leukocyte count usually abo*e 78)888"9!. &ay in*ol*e neutrophils) lymphocytes) or eosinophils b. Etiology ii Perforating appendicitis (neutrophils+ iii :hooping cough (lymphocytes+ iiii Cutaneous lar*a migrans (eosinophils+ b. Pathogenesis Exaggerated response to infection ii !eukoerythroblastic reaction a. 5mmature bone marro. cells enter the peripheral blood b. Pathogenesis ii $one marro. infiltrati*e disease iii Examples-fibrosis) metastatic breast cancer b. Peripheral blood findings ii &yeloblasts) progranulocytes iii ;ucleated <$Cs) tear drop <$Cs Benign Quantitative WBC Disorders Disorders in*ol*ing neutrophils page # page = 1. ;eutrophilic leukocytosis a. 6bsolute neutrophil count abo*e >888"9! b. Etiology i. 5nfection (e.g.) acute appendicitis+ ii. 2terile inflammation .ith necrosis (e.g.) acute myocardial infarction+ iii. Drugs (e.g.) corticosteroids+ c. Pathogenesis i. 5ncreased bone marro. production or release of neutrophils ii. Decreased acti*ation of neutrophil adhesion molecules !ess neutrophils adhere to endothelial cells Examples-corticosteroids) catecholamines) lithium . ;eutropenia a. 6bsolute neutrophil count belo. 1788"9! b. Etiology i. 6plastic anemia ii. 5mmune destruction Example-systemic lupus erythematosus (2!E+ iii. 2eptic shock c. Pathogenesis i. Decreased production ii. 5ncreased destruction (e.g.) complement) macrophages+ iii. 6cti*ation of neutrophil adhesion molecules (e.g.) endotoxins+ 5ncrease the number of neutrophils adhering to endothelium Disorders in*ol*ing neutrophils page # page = 1. ;eutrophilic leukocytosis a. 6bsolute neutrophil count abo*e >888"9! b. Etiology i. 5nfection (e.g.) acute appendicitis+ ii. 2terile inflammation .ith necrosis (e.g.) acute myocardial infarction+ iii. Drugs (e.g.) corticosteroids+ c. Pathogenesis i. 5ncreased bone marro. production or release of neutrophils ii. Decreased acti*ation of neutrophil adhesion molecules !ess neutrophils adhere to endothelial cells Examples-corticosteroids) catecholamines) lithium . ;eutropenia a. 6bsolute neutrophil count belo. 1788"9! b. Etiology i. 6plastic anemia ii. 5mmune destruction Example-systemic lupus erythematosus (2!E+ iii. 2eptic shock c. Pathogenesis i. Decreased production ii. 5ncreased destruction (e.g.) complement) macrophages+ iii. 6cti*ation of neutrophil adhesion molecules (e.g.) endotoxins+ 5ncrease the number of neutrophils adhering to endothelium Disorders in*ol*ing eosinophils 1. Eosinophilia a. 6bsolute eosinophil count o*er >88"9! b. Etiology i. ?ype 5 hypersensiti*ity reaction Examples-bronchial asthma) reaction to penicillin) hay fe*er ii. 5n*asi*e helminthic infection Examples-strongyloidiasis) hook.orm infection Pin.orms and adult ascariasis do not ha*e eosinophilia (nonin*asi*e+. iii. Polyarteritis nodosa) 6ddison%s disease (cortisol deficiency+ c. Pathogenesis i. <elease of eosinophil chemotactic factor from mast cells (e.g.) type 5 hypersensiti*ity+ ii. ;o se-uestering of eosinophils in lymph nodes (e.g.) hypocortisolism+ . Eosinopenia a. Hypercortisolism (e.g.) Cushing syndrome) corticosteroids+ b. Corticosteroids se-uester eosinophils in lymph nodes. Disorders in*ol*ing basophils@ basophilia 1. 6bsolute basophil count o*er 118"9! . Etiology o Chronic myeloproliferati*e disorders (e.g.) polycythemia *era+ Disorders in*ol*ing lymphocytes page /1 1. !ymphocytosis a. 6bsolute lymphocyte count o*er 1888"9! in adults or o*er #888"9! in children b. Etiology i. Airal (e.g.) mononucleosis+ or bacterial (e.g.) .hooping cough+ ii. Drugs (e.g.) phenytoin+ iii. Bra*es% disease c. Pathogenesis i. 5ncreased production ii. Decreased entry into lymph nodes Example-lymphocytosis-promoting factor produced by Bordetella pertussis . 6typical lymphocytosis a. Etiology i. 5nfection Examples-mononucleosis) *iral hepatitis) cytomegalo*irus infection) toxoplasmosis ii. Drugs (e.g.) phenytoin+ b. Pathogenesis i. 6ntigenically stimulated lymphocytes ii. Prominent nucleoli and abundant blue cytoplasm /. 5nfectious mononucleosis a. Caused by Epstein-$arr *irus (E$A+ b. Pathogenesis i. Primarily transmitted by kissing E$A initially replicates in the sali*ary glands and then disseminates. ii. E$A attaches to CD1 receptors on $ cells. Causes $-cell proliferation and increased synthesis of antibodies iii. Airus remains dormant in $ cells. <ecurrences may occur. c. Clinical findings i. ,atigue) tonsillitis ii. Hepatosplenomegaly) generali4ed lymphadenopathy Danger of splenic rupture in contact sports iii. <ash de*elops if treated .ith ampicillin. d. !aboratory findings i. 6typical lymphocytosis 'sually more than 8C of the total :$C count 6typical lymphocytes are antigenically stimulated ? cells ii. Positi*e heterophil antibody test Detects 5g& antibodies against horse (most common+) sheep) and bo*ine <$Cs iii. Positi*e anti*iral capsid antigen test &ost sensiti*e test i*. 5ncreased serum transaminases from hepatitis 3aundice is rare. 1. !ymphopenia a. 6bsolute lymphocyte count belo. 1788"9! in adults or belo. /888"9! in children b. Etiology i. Human immunodeficiency *irus (H5A+ ii. 5mmunodeficiency DiBeorge syndrome (?-cell deficiency+ 2e*ere combined immunodeficiency ($- and ?-cell deficiency+ iii. 5mmune destruction (e.g.) 2!E+ i*. Corticosteroids *. <adiation !ymphocytes are the most sensiti*e cells to destruction by radiation. c. Pathogenesis i. 5ncreased destruction Examples-lysis of CD1 helper ? cells by the *irus@ apoptosis by corticosteroids@ immune destruction ii. Decreased production Example-radiation iii. Decreased release from lymph nodes Example-corticosteroids Disorders in*ol*ing monocytes@ monocytosis page /1 page / 1. 6bsolute monocyte count o*er #88"9! . Etiology a. Chronic infection (e.g.) tuberculosis+ b. 6utoimmune disease (e.g.) rheumatoid arthritis+ c. &alignancy (e.g.) carcinoma) malignant lymphoma+ /. Pathogenesis o <esponse to chronic inflammation or malignancy Leukemias (Acute and Chronic) Epidemiology 1. &alignant diseases of bone marro. stem cells that may in*ol*e all cell lines . <isk factors a. Chromosomal abnormalities Examples-Do.n syndrome) chromosome instability syndromes b. 5oni4ing radiation c. Chemicals (e.g.) ben4ene+ d. 6lkylating agents (particularly busulfan+ /. 6ge ranges for common leukemias a. ;e.born to 11 years old 6cute lymphoblastic leukemia (6!!+ b. Persons 17 to /= years old 6cute myelogenous leukemia (6&!+ c. Persons 18 to D8 years old ii 6&! (ED8C of cases+ iii Chronic myelogenous leukemia (18C of cases+ b. Persons o*er D8 years of age Chronic lymphocytic leukemia (C!!+ Epidemiology 1. &alignant diseases of bone marro. stem cells that may in*ol*e all cell lines . <isk factors a. Chromosomal abnormalities Examples-Do.n syndrome) chromosome instability syndromes b. 5oni4ing radiation c. Chemicals (e.g.) ben4ene+ d. 6lkylating agents (particularly busulfan+ /. 6ge ranges for common leukemias a. ;e.born to 11 years old 6cute lymphoblastic leukemia (6!!+ b. Persons 17 to /= years old 6cute myelogenous leukemia (6&!+ c. Persons 18 to D8 years old ii 6&! (ED8C of cases+ iii Chronic myelogenous leukemia (18C of cases+ b. Persons o*er D8 years of age Chronic lymphocytic leukemia (C!!+ Pathogenesis 1. $lock in stem cell differentiation o &onoclonal proliferation of neoplastic leukocytes behind the block . !eukemic cells a. <eplace the bone marro. <eplace normal hematopoietic cells b. Enter the peripheral blood c. &etastasi4e throughout the body Clinical findings in acute leukemia 1. 6brupt onset of signs and symptoms . ,e*er (infection+) bleeding (thrombocytopenia+) fatigue (anemia+ /. &etastatic disease a. Hepatosplenomegaly b. Benerali4ed lymphadenopathy c. Central ner*ous system (C;2+ in*ol*ement (especially in 6!!+ d. 2kin in*ol*ement (especially ?-cell leukemias+ 1. $one pain and tenderness o Due to bone marro. expansion by leukemic cells !aboratory findings in acute leukemia page / page // 1. Peripheral :$C count a. $elo. 18)888"9! (normal+ to more than 188)888"9! b. $last cells usually more than 8C (e.g.) myeloblasts) lymphoblasts+. . ;ormocytic to macrocytic anemia o &acrocytic if folate is depleted in production of leukemic cells 2. ?hrombocytopenia (usually F188)888"9!+ 3. $one marro. findings o Hypercellular .ith more than 8C blasts (e.g.) myeloblasts) lymphoblasts+ Clinical findings in chronic leukemia a. 5nsidious onset b. Hepatosplenomegaly and generali4ed lymphadenopathy !aboratory findings in chronic leukemia a. Peripheral :$C count i. 2imilar to that of acute leukemia ii. $last cells usually less than 18C iii. E*idence of maturation of cells b. ;ormocytic to macrocytic anemia o &acrocytic if folate is depleted in production of leukemic cells b. ?hrombocytopenia (usually F188)888"9!+ o Exception in C&!) in .hich thrombocytosis occurs in 18C of cases c. $one marro. findings o Hypercellular .ith less than 18C blasts eoplastic Meloid Disorders G*er*ie. 1. &yeloid disorders are neoplastic stem cell disorders. o &ay in*ol*e one or more stem cell lines . Classification a. Chronic myeloproliferati*e disorders b. &yelodysplastic syndrome c. 6cute myeloblastic leukemia G*er*ie. 1. &yeloid disorders are neoplastic stem cell disorders. o &ay in*ol*e one or more stem cell lines . Classification a. Chronic myeloproliferati*e disorders b. &yelodysplastic syndrome c. 6cute myeloblastic leukemia Chronic myeloproliferati*e disorders !a"le 12#1$ La"orator %indings in &olcthemias &olcthemia 'BC &lasma (a)2 *&) Mass +olume Polycythemia *era ;ormal 6ppropriate polycythemia (e.g.) CGPD) cyanotic congenital heart disease+ ;ormal 5nappropriate polycythemiaH ectopic EPG (e.g.) renal disease+ ;ormal ;ormal <elati*e polycythemia (e.g.) *olume depletion+ ;ormal ;ormal ;ormal CGPD) chronic obstructi*e pulmonary disease@ EPG) erythropoietin@ 2aG) oxygen saturation. page /D page /> 1. Classification a. Polycythemia *era b. Chronic myelogenous leukemia c. &yeloid metaplasia .ith myelofibrosis d. Essential thrombocythemia . Beneral characteristics a. 2plenomegaly b. Propensity for reacti*e bone marro. fibrosis (0spent phase0+ c. Propensity for transformation to acute leukemia /. Polycythemia a. 5ncreased hemoglobin (Hb+) hematocrit (Hct+) and <$C count b. Plasma *olume (PA+ *aries .ith the type of polycythemia. c. <$C count *ersus <$C mass i. <$C count is the number of <$Cs per 9! of blood. ii. <$C mass is the total number of <$Cs in the body in m!"kg. iii. <$C count is the ratio of <$C mass to plasma *olume (PA+. sho.s the normal relationship bet.een <$C count) <$C mass) PA) erythropoietin (EPG+) and G saturation (2ao+. d. <elati*e polycythemia i. 5ncreased <$C count due to a decrease in PA Example-*olume depletion from s.eating ii. <$C mass is normal. No increase in bone marro. production of <$Cs iii. Erythropoietin (EPG+ and 2ao are normal. e. 6bsolute polycythemia i. 5ncrease in bone marro. production of <$Cs 5ncreased <$C count and <$C mass ii. 6ppropriate absolute polycythemia if there is a hypoxic stimulus for EPG release Examples-primary lung disease) cyanotic congenital heart disease) li*ing at high altitude Decreased G saturation (2ao+ 5ncreased <$C count) <$C mass) EPG ;ormal PA iii. 5nappropriate absolute polycythemia if there is no hypoxic stimulus for EPG release Polycythemia *era (see belo.+ Ectopic secretion of EPG (e.g.) renal cell carcinoma+ 5ncreased <$C count) <$C mass) EPG@ normal PA and 2ao 1. Polycythemia *era a. Pathogenesis i. Clonal expansion of the trilineage myeloid stem cell ii. 5ncrease in <$Cs) granulocytes (neutrophils) eosinophils) basophils+) mast cells) and platelets b. Clinical findings i. 2plenomegaly ii. ?hrombotic e*ents due to hyper*iscosity (e.g.) hepatic *ein thrombosis+ iii. 2igns of increased histamine (released from mast cells in the skin+ <uddy face Pruritus after bathing Peptic ulcer disease (histamine stimulates production of gastric acid+ i*. Bout Due to increased breakdo.n of nucleated cells .ith release of purines (con*erted to uric acid+ c. !aboratory findings in polycythemia *era i. 5ncreased <$C mass and PA Gnly type of polycythemia .ith an increase in PA ii. 6bsolute leukocytosis (leukocytes E1)888"9!+ iii. ?hrombocytosis (platelets E188)888"9!+ i*. Decreased EPG 5ncreased G content inhibits EPG release. Gnly type of polycythemia .ith decreased EPG *. ;ormal 2ao *i. Hypercellular bone marro. .ith fibrosis in later stages d. 2ummary table of the polycythemias 7. Chronic myelogenous leukemia (C&!+ a. Epidemiology i. 'sually occurs bet.een 18 and D8 years of age ii. <isk factors Exposure to ioni4ing radiation and ben4ene b. Pathogenesis i. ;eoplastic clonal expansion of the pluripotential stem cell ?his stem cell has the capacity to differentiate into a lymphoid or trilineage myeloid stem cell. ii. t=@ translocation of ABL proto-oncogene Proto-oncogene fuses .ith the break cluster region ($C<+ on chromosome (BCR-ABL fusion gene+. c. Clinical findings i. Hepatosplenomegaly and generali4ed lymphadenopathy Due to metastasis ii. $last crisis 'sually occurs in 7 years 5ncrease in numbers of myeloblasts or lymphoblasts &yeloblasts do not contain 6uer rods (see belo.+ d. !aboratory findings i. Peripheral :$C count 78)888 to 88)888 cells"9! &yeloid series in all stages of de*elopment ii. ;ormocytic to macrocytic anemia &acrocytic if folate is depleted in the production of leukemic cells. iii. Platelet count ?hrombocytosis (18-78C+) thrombocytopenia in the remainder of cases i*. $one marro. findings &yeloblasts less than 18C Hypercellular *. Positi*e Philadelphia chromosome (=7C of cases+ 5t is not specific for C&! and is present in other leukemias. 5t is not lost during therapy unless I-interferon is used. *i. BCR-ABL fusion gene (188C of cases+ ,usion gene is the most sensiti*e and specific test for C&! *ii. Decreased leukocyte alkaline phosphatase (!6P+ !6P is absent in neoplastic granulocytes and present in benign granulocytes. D. &yelofibrosis and myeloid metaplasia a. Pathogenesis i. &arro. fibrosis occurs earlier than in other types of myeloproliferati*e disease. ii. ;eoplastic cells are produced in the spleen and other sites (extramedullary hematopoiesis) E&H+. b. Clinical findings i. &assi*e splenomegaly .ith portal hypertension ii. 2plenic infarcts .ith left-sided pleural effusions c. !aboratory findings i. $one marro. fibrosis due to stimulation of fibroblasts ii. Peripheral :$C count 18)888 to 78)888 cells"9! iii. ;ormocytic anemia ?ear-drop cells (damaged <$Cs+ !eukoerythroblastic reaction i*. Platelet count is *ariable (increased or decreased+. >. Essential thrombocythemia a. Pathogenesis i. ;eoplastic stem cell disorder .ith proliferation of megakaryocytes ii. Platelets are increased@ ho.e*er) they are nonfunctional. b. Clinical findings i. $leeding (usually gastrointestinal .ith concomitant iron deficiency+ ii. 2plenomegaly c. !aboratory findings i. ?hrombocytosis (platelets ED88)888"9!+ Platelet morphology is abnormal. ii. &ild neutrophilic leukocytosis iii. Hypercellular bone marro. .ith abnormal megakaryocytes &yelodysplastic syndrome page /> page /# 1. Epidemiology o 'sually occurs in men bet.een 78 and #8 years old . Pathogenesis a. Broup of neoplastic stem cell disorders Chromosomal abnormalities in 78C of cases (e.g.) 7- - ) trisomy #+ b. ,re-uently progresses to acute myelogenous leukemia (6&!@ /8C of cases+ 0Preleukemia0 . !aboratory findings a. 2e*ere pancytopenia ii ;ormocytic to macrocytic anemia Dimorphic <$C population (microcytic and macrocytic+ iii !eukoerythroblastic reaction b. $one marro. findings ii <inged sideroblasts (nucleated <$Cs .ith excess iron+ iii &yeloblasts less than 8C (if E8C) disease is progressing to 6&!+ 6cute myelogenous leukemia !a"le 12#2$ %rench#American#British Classi,ication o, Acute Melogenous Leukemia (AML) Class Comments &8H &inimally differentiated 6&! ;o 6uer rods &1H 6&! .ithout differentiationH 8C <are 6uer rods &H 6&! .ith maturation &ost common type (/8-18C of cases+. 6uer rods present 17-7=-year-old age bracket &/H 6cute promyelocytic ;umerous 6uer rods D5C is in*ariably present t(17@1>+ translocation 6bnormal retinoic acid metabolismH high doses of *itamin 6 may induce remission by maturing cells &1H 6cute myelomonocytic 6uer rods uncommon &7H 6cute monocytic ;o 6uer rods Bum infiltration &DH 6cute erythroleukemia $i4arre) multinucleated erythroblasts &yeloblasts present &>H 6cute megakaryocytic &yelofibrosis in bone marro. 5ncreased incidence in Do.n syndrome in children F/ years old D5C) disseminated intra*ascular coagulation. 1. Epidemiology a. 'sually occurs bet.een 17 and 7= years of age b. ,rench-6merican-$ritish (,6$+ classification is used . Cytogenetic abnormalities are common. o Example-t(17@1>+ in acute promyelocytic leukemia (&/+ 2. Clinical findings i. Disseminated intra*ascular coagulation (D5C+ is common. 5n*ariable in acute promyelocytic leukemia ii. Bum infiltration is common in acute monocytic leukemia (&7+. . 6uer rods i. 2plinter-shaped to rod-shaped structures in the cytosol of myeloblasts 6uer rods are fused a4urophilic granules ii. Gnly present in acute myelogenous leukemia (& and &/+ ?hey are not present in myeloblasts in chronic myelogenous leukemia. Lmphoid Leukemias 6cute lymphoblastic leukemia (6!!+ 1. Epidemiology a. &ost common leukemia in children (ne.born to 11 years of age+ b. 2ubtypes i. Early pre-$-cell 6!! (#8C+ ii. Pre-$-) $-) and ?-cell 6!! . Pathogenesis o Clonal lymphoid stem cell disease 2. Early pre-$-cell 6!! a. Positi*e marker studies for common 6!! antigen (C6!!6) CD18+ b. Positi*e marker studies for terminal deoxynucleotidyl transferase (?d?+ c. t(1@1+ translocation offers a fa*orable prognosis. d. Breater than =8C achie*e complete remission. 6t least t.o thirds of patients can be considered cured. . ?-cell 6!! o CD18 negati*e and ?d? positi*e . Clinical findings o &etastatic sites similar to those of 6&! o $-cell types Commonly metastasi4e to the C;2 and testicles o ?-cell type Presents as anterior mediastinal mass or acute leukemia /. !aboratory findings o Peripheral :$C count 18)888 to 188)888"9! G*er 8C lymphoblasts in peripheral blood o ;ormocytic anemia .ith thrombocytopenia o $one marro. findings $one marro. often totally replaced by lymphoblasts 6cute lymphoblastic leukemia (6!!+ 1. Epidemiology a. &ost common leukemia in children (ne.born to 11 years of age+ b. 2ubtypes i. Early pre-$-cell 6!! (#8C+ ii. Pre-$-) $-) and ?-cell 6!! . Pathogenesis o Clonal lymphoid stem cell disease 2. Early pre-$-cell 6!! a. Positi*e marker studies for common 6!! antigen (C6!!6) CD18+ b. Positi*e marker studies for terminal deoxynucleotidyl transferase (?d?+ c. t(1@1+ translocation offers a fa*orable prognosis. d. Breater than =8C achie*e complete remission. 6t least t.o thirds of patients can be considered cured. . ?-cell 6!! o CD18 negati*e and ?d? positi*e . Clinical findings o &etastatic sites similar to those of 6&! o $-cell types Commonly metastasi4e to the C;2 and testicles o ?-cell type Presents as anterior mediastinal mass or acute leukemia /. !aboratory findings o Peripheral :$C count 18)888 to 188)888"9! G*er 8C lymphoblasts in peripheral blood o ;ormocytic anemia .ith thrombocytopenia o $one marro. findings $one marro. often totally replaced by lymphoblasts 6dult ?-cell leukemia 1. Epidemiology a. &alignant leukemia associated .ith human ?-cell leukemia *irus (H?!A-1+ b. &ay present as a malignant lymphoma . Pathogenesis a. 6cti*ation of TAX gene) .hich inhibits the TP! suppressor gene b. !eads to monoclonal proliferation of neoplastic CD1 helper ? cells /. Clinical findings a. Hepatosplenomegaly and generali4ed lymphadenopathy b. 2kin infiltration Common finding in all ?-cell malignancies c. !ytic bone lesions ii Due to lymphoblast release of osteoclast-acti*ating factor iii 6ssociated .ith hypercalcemia . !aboratory findings a. Peripheral :$C count 18)888 to 78)888"9! ii G*er 8C lymphoblasts iii Positi*e CD1 marker study iiii ;egati*e for ?d? b. ;ormocytic anemia and thrombocytopenia c. $one marro. findings <eplaced by CD1 lymphoblasts Chronic lymphocytic leukemia (C!!+ 1. Epidemiology a. Gccurs in indi*iduals o*er D8 years old b. &ost common o*erall leukemia c. &ost common cause of generali4ed lymphadenopathy in the same age bracket . Pathogenesis o ;eoplastic disorder of *irgin $ cells ($ cells that cannot differentiate into plasma cells+ 2. Clinical findings a. Benerali4ed lymphadenopathy b. &etastatic sites similar to those of 6&! c. 5ncreased incidence of immune hemolytic anemia $oth .arm (5gB+ and cold (5g&+ types /. !aboratory findings d. Peripheral :$C count 17)888 to 88)888"9! ii !ymphoblasts less than 18C iii ;eutropenia iiii ;umerous 0smudge0 cells (fragile leukemic cells+ b. ;ormocytic anemia (78C of cases+ and thrombocytopenia (18C of cases+ c. $one marro. findingsH usually replaced by neoplastic $ cells d. Hypogammaglobulinemia is common. Hairy cell leukemia 1. ?ype of $-cell leukemia o &ost common in middle-aged men . Clinical findings a. 2plenomegaly (=8C of cases+ b. 6bsence of lymphadenopathy Gnly leukemia "ithout lymphadenopathy c. Hepatomegaly (8C of cases+ d. 6utoimmune *asculitis and arthritis . !aboratory findings a. Pancytopenia !eukemic cells ha*e hair-like proJections b. Positi*e tartrate-resistant acid phosphatase stain (?<6P+ 2ummary table of the lymphoid leukemias !a"le 12#-$ (ummar o, Acute and Chronic Lmphoid Leukemias Leukemia Description 6cute lymphoblastic &ost common leukemia in children (Early pre-$ type+ ;e.born to 11 years old C6!!6 (CD18+ and ?d? positi*e t(1@1+ offers a good prognosis Chronic lymphocytic Airgin $ cell leukemia Patients E D8 years old &ost common cause of generali4ed lymphadenopathy in same age bracket Hypogammaglobulinemia 6dult ? cell H?!A-1 association !eukemic cells CD1 positi*e and ?d? negati*e 2kin infiltration !ytic bone lesions .ith hypercalcemia Hairy cell $ cell leukemia Cytoplasmic proJections ?<6P stain positi*e 2plenomegaly 6bsence of lymphadenopathy Pancytopenia Dramatic response to purine nucleosides C6!!6) common acute lymphoblastic leukemia antigen@ H?!A) human ?-cell leukemia@ ?d?) terminal deoxynucleotidyl transferase@ ?<6P) tartrate resistant acid phosphatase.