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1542  Part XI:  Malignant Lymphoid Diseases                    Chapter 92:  Chronic Lymphocytic Leukemia             1543




                  men and increases in incidence with age. The cumulative annual risk of   immature B cells with distinct molecular features and outcomes (Chap.
                  patients with MBL who require CLL-specific therapy is approximately   91). Transformation of CLL into ALL has been reported and managed
                  1 percent. 16,17,420  Retrospective analysis of samples collected on longi-  with regimens suitable for ALL. 437–439
                  tudinal, prospective studies have also demonstrated that the develop-
                  ment of overt CLL is almost always preceded by MBL.  Management
                                                         423
                  of patients with MBL is similar to patients with early stage CLL and   REFERENCES
                  the vast majority of these patients can be followed with watchful wait-    1.  Hallek M, Cheson BD, Catovsky D, et al: Guidelines for the diagnosis and treatment of
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                  quency of infections and also secondary malignancies mandating follow   Lymphocytic Leukemia updating the National Cancer Institute-Working Group 1996
                  up similar to that recommended for CLL. Our own practice is to see   guidelines. Blood 111:5446–5456, 2008.
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                  and mimics it with regards to clinical course and outcomes. The malig-    5.  Ahn YO, Koo HH, Park BJ, et al: Incidence estimation of leukemia among Koreans.
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                  analogous to CLL. 1,45,424,425                          8.  Wang SS, Slager SL, Brennan P, et al: Family history of hematopoietic malignancies and
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                                                                           controls from the International Lymphoma Epidemiology Consortium (InterLymph).
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                  DIFFERENTIAL DIAGNOSES                                  9.  Slager SL, Benavente Y, Blair A, et al: Medical history, lifestyle, family history, and occu-
                                                                           pational risk factors for chronic lymphocytic leukemia/small lymphocytic lymphoma:
                  Multiple low-grade lymphoid malignancies can mimic the presentation   The InterLymph Non-Hodgkin Lymphoma Subtypes Project. J Natl Cancer Inst Monogr
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                  and laboratory evidence of CLL and are discussed in detail in the chap-    10.  Marwick C: Link found between Agent Orange and chronic lymphocytic leukaemia.
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                  MANTLE CELL LYMPHOMA                                    12.  Feychting M, Forssen U, Floderus B: Occupational and residential magnetic field expo-
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                  Mantle cell lymphoma frequently mimics the clinical presentation and     13.  Vrijheid M, Cardis E, Ashmore P, et al: Ionizing radiation and risk of chronic lym-
                  laboratory findings observed in patients with CLL (Chap. 100). How-  phocytic leukemia in the 15-country study of nuclear industry workers. Radiat Res
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                  ever,  the  malignant  lymphocytes  observed  in  mantle  cell  lymphoma     14.  Richardson DB, Wing S, Schroeder J, et al: Ionizing radiation and chronic lymphocytic
                  express CD5 without coexpression of CD23 and have a higher level of   leukemia. Environ Health Perspect 113:1–5, 2005.
                  expression of CD79. Cases of CD23-negative CLL and CD23-positive     15.  Abramenko I, Bilous N, Chumak A, et al: Chronic lymphocytic leukemia patients
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                  ing feature between the two disorders is the presence of t(11;14) in man-    16.  Rawstron AC, Yuille MR, Fuller J, et al: Inherited predisposition to CLL is detectable as
                  tle cell lymphoma, which results in the bcl-1-IGH translocation and the   subclinical monoclonal B-lymphocyte expansion. Blood 100:2289–2290, 2002.
                  overexpression of cyclin D1. Therapy for mantle cell lymphoma employs     17.  Rawstron AC, Bennett FL, O’Connor SJ, et al: Monoclonal B-cell lymphocytosis and
                                                                           chronic lymphocytic leukemia. N Engl J Med 359:575–583, 2008.
                  some of the agents used for CLL but is covered in detail elsewhere. 426–429    18.  Kristinsson SY, Bjorkholm M, Goldin LR, et al: Risk of lymphoproliferative disorders
                                                                           among first-degree relatives of lymphoplasmacytic lymphoma/Waldenstrom macro-
                                                                           globulinemia patients: A population-based study in Sweden.  Blood 112:3052–3056,
                  HAIRY CELL LEUKEMIA                                      2008.
                                                                          19.  Raval A, Tanner SM, Byrd JC, et al: Downregulation of death-associated protein kinase
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                  the presence of typical villous “hairy” projections from the surface of its     20.  Perez-Chacon G, Contreras-Martin B, Cuni S, et al: Polymorphism in the CD5 gene
                  cells (Chap. 93). The cells are also strongly positive for tartrate-resistant   promoter in B-cell chronic lymphocytic leukemia and mantle cell lymphoma. Am J Clin
                  acid phosphatase (TRAP) and express CD11c, CD25, and CD103. With   Pathol 123:646–650, 2005.
                  the discovery of the BRAF V600E  in HCL, significant improvements have     21.  Aydin S, Rossi D, Bergui L, et al: CD38 gene polymorphism and chronic lymphocytic
                                                                           leukemia: A role in transformation to Richter syndrome? Blood 111:5646–5653, 2008.
                  been made in the availability of therapeutic agents for the management    22.  Jevtovic-Stoimenov T, Kocic G, Pavlovic D, et al: Polymorphisms of tumor-necrosis
                  of these patients. 430–433                               factor-alpha -308 and lymphotoxin-alpha + 250: Possible modulation of susceptibility
                                                                           to apoptosis in chronic lymphocytic leukemia and non-Hodgkin lymphoma mononu-
                                                                           clear cells. Leuk Lymphoma 49:2163–2169, 2008.
                  OTHER LYMPHOID MALIGNANCIES                             23.  Sellick GS, Goldin LR, Wild RW, et al: A high-density SNP genome-wide linkage search
                                                                           of 206 families identifies susceptibility loci for chronic lymphocytic leukemia. Blood
                  Various low-grade lymphoid malignancies can mimic the clinical and   110:3326–3333, 2007.
                  laboratory presentation of CLL and include marginal zone lymphomas     24.  Di Bernardo MC, Crowther-Swanepoel D, Broderick P, et al: A genome-wide associa-
                  and lymphoplasmacytic lymphomas. However, pathologic evaluation   tion study identifies six susceptibility loci for chronic lymphocytic leukemia. Nat Genet
                                                                           40:1204–1210, 2008.
                  results in the identification of surface markers CD19 and CD20 without     25.  Marti GE, Faguet G, Bertin P, et al: CD20 and CD5 expression in B-chronic lympho-
                  coexpression of CD5 or CD23. Therapy is directed at the specific lym-  cytic leukemia. Ann N Y Acad Sci 651:480–483, 1992.
                  phoid malignancy identified. 434–436  B-cell ALL is also included in the list     26.  Almasri NM, Duque RE, Iturraspe J, et al: Reduced expression of CD20 antigen as a
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          Kaushansky_chapter 92_p1527-1552.indd   1543                                                                  9/18/15   10:49 AM
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