*6.1.3 Treatment and prognosis*

Treatment includes chemotherapy, steroids, radiation therapy and marrow transplantation. About 95% of children obtain a complete remission, and 75–85% are cured


*Mature B-cell neoplasms*

#### **WHO classification of B lymphoid neoplasms**

Monoclonal B-cell lymphocytosis\* B-cell prolymphocytic leukemia Splenic marginal zone lymphoma Hairy cell leukemia Splenic B-cell lymphoma/leukemia, unclassifiable Splenic diffuse red pulp small B-cell lymphoma Hairy cell leukemia-variant Lymphoplasmacytic lymphoma Waldenstro¨m macroglobulinemia Monoclonal gammopathy of undetermined significance (MGUS), IgM\* m heavy-chain disease g heavy-chain disease a heavy-chain disease Monoclonal gammopathy of undetermined significance (MGUS), IgG/A\* Plasma cell myeloma Solitary plasmacytoma of bone Extraosseous plasmacytoma Monoclonal immunoglobulin deposition diseases\* Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) Nodal marginal zone lymphoma Pediatric nodal marginal zone lymphoma Follicular lymphoma In situ follicular neoplasia\* Duodenal-type follicular lymphoma\* Pediatric-type follicular lymphoma\* Large B-cell lymphoma with IRF4 rearrangement\* Primary cutaneous follicle center lymphoma Mantle cell lymphoma In situ mantle cell neoplasia\* Diffuse large B-cell lymphoma (DLBCL), NOS Germinal center B-cell type\* Activated B-cell type\* T-cell/histiocyte-rich large B-cell lymphoma Primary DLBCL of the central nervous system (CNS) Primary cutaneous DLBCL, leg type EBV1 DLBCL, NOS\* EBV1 mucocutaneous ulcer\* DLBCL associated with chronic inflammation Lymphomatoid granulomatosis Primary mediastinal (thymic) large B-cell lymphoma Intravascular large B-cell lymphoma ALK1 large B-cell lymphoma Plasmablastic lymphoma Primary effusion lymphoma HHV81 DLBCL, NOS\* Burkitt lymphoma Burkitt-like lymphoma with 11q aberration\* High-grade B-cell lymphoma, with MYC and BCL2 and/or BCL6 rearrangements\* High-grade B-cell lymphoma, NOS\* B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and classical Hodgkin lymphoma

*\*Changes from the 2008 classification.*

#### **Table 1.**

*WHO classification of B lymphoid neoplasms*

with chemotherapy, whereas only 35–40% of adults are cured. Treatment of t(9;22) positive ALLs with BCR-ABL kinase inhibitors in combination with conventional chemotherapy is highly effective and has greatly improved the outcome in children. The outlook for adults with ALL remains more guarded, in part because of differences

**53**

mm3

**Figure 1.**

*B-Cell Lymphomas*

*DOI: http://dx.doi.org/10.5772/intechopen.87370*

*B-lymphoblastic lymphoma showing CD20, Tdt positivity.*

in the molecular pathogenesis of adult and childhood ALL, but also because older adults cannot tolerate the very intensive chemotherapy regimens that are curative in children. Factors associated with worse prognosis includes: (1) age younger than 2 years, (2) presentation in adolescence or adulthood; and (3) peripheral blood blast counts greater than 100,000. Factors associated with favorable prognosis include (1) age between 2 and 10 years, (2) a low white cell count, (3) hyperdiploidy, (4) trisomy

Chronic lymphocytic leukemia (CLL) and small lymphocytic lymphoma (SLL) are used interchangeably by the degree of peripheral blood lymphocytosis. CLL is the most common leukemia of adults with absolute lymphocyte count >5000 per

. The median age at diagnosis is 60 years. SLL constitutes only 4% of NHLs and has lymph node involvement The common genetic anomalies associated are deletions of 13q14.3, 11q, and 17p, and trisomy 12q [1, 25]. Patients are usually asymptomatic at diagnosis and can present with easy fatigability, weight loss, and anorexia. Generalized lymphadenopathy and hepatosplenomegaly are present in 50–60% of patients. The leukocyte count is high in most cases; leukopenia can be

Peripheral blood contains large numbers of small round lymphocytes with scant cytoplasm. Some of these cells are usually disrupted in the process of making smears, producing smudge cells. Prolymphocytes can also be circulating based on which it is classified as CLL or CLL/PLL or PLL (Prolymphocytic leukemia). The bone marrow is almost always involved by interstitial infiltrates or aggregates of tumor cells. Infiltrates are also virtually always seen in the splenic white and red pulp and the hepatic portal tracts. Lymph nodes are diffusely effaced by predominantly small lymphocytes with mild irregular nucleus, condensed chromatin, and scant cytoplasm along with proliferation centers, which contain aggregates of mitotically active larger activated lymphocytes. (**Figure 2**).CLL/SLL has a distinctive immunophenotype. The tumor cells express the pan B-cell markers CD19 and CD20, CD23, CD5, LEF1 (Lymphoid Enhancer Binding Factor 1), CD200. Negative for SOX11 (SRY (sex determining region) - Box 11), CD10.

Cytopenias or disease-related symptoms are now insufficient to make a diagno-

of chromosomes 4, 7, and 10, and (5) the presence of a t(12;21) [1, 25, 38].

**6.2 Chronic lymphocytic leukemia/small lymphocytic lymphoma**

seen in individuals with SLL and marrow involvement.

*6.2.1 Morphology and immunophenotype*

*6.2.2 Highlights in recent WHO classification*

/L PB CLL cells.

sis of CLL with <5 × 109

*Normal and Malignant B-Cell*

Hairy cell leukemia

Hairy cell leukemia-variant Lymphoplasmacytic lymphoma Waldenstro¨m macroglobulinemia

m heavy-chain disease g heavy-chain disease a heavy-chain disease

Plasma cell myeloma Solitary plasmacytoma of bone Extraosseous plasmacytoma

marginal zone lymphoma

Follicular lymphoma

Mantle cell lymphoma In situ mantle cell neoplasia\*

EBV1 DLBCL, NOS\* EBV1 mucocutaneous ulcer\*

Monoclonal B-cell lymphocytosis\* B-cell prolymphocytic leukemia Splenic marginal zone lymphoma

**WHO classification of B lymphoid neoplasms**

Splenic B-cell lymphoma/leukemia, unclassifiable Splenic diffuse red pulp small B-cell lymphoma

Monoclonal immunoglobulin deposition diseases\*

Large B-cell lymphoma with IRF4 rearrangement\* Primary cutaneous follicle center lymphoma

Diffuse large B-cell lymphoma (DLBCL), NOS Germinal center B-cell type\* Activated B-cell type\* T-cell/histiocyte-rich large B-cell lymphoma Primary DLBCL of the central nervous system (CNS)

DLBCL associated with chronic inflammation

Burkitt-like lymphoma with 11q aberration\*

High-grade B-cell lymphoma, NOS\*

*WHO classification of B lymphoid neoplasms*

*\*Changes from the 2008 classification.*

lymphoma

**Table 1.**

Primary mediastinal (thymic) large B-cell lymphoma

Pediatric nodal marginal zone lymphoma

In situ follicular neoplasia\* Duodenal-type follicular lymphoma\*

Pediatric-type follicular lymphoma\*

Primary cutaneous DLBCL, leg type

Lymphomatoid granulomatosis

Intravascular large B-cell lymphoma ALK1 large B-cell lymphoma Plasmablastic lymphoma Primary effusion lymphoma HHV81 DLBCL, NOS\* Burkitt lymphoma

Monoclonal gammopathy of undetermined significance (MGUS), IgM\*

Monoclonal gammopathy of undetermined significance (MGUS), IgG/A\*

Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) Nodal

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with chemotherapy, whereas only 35–40% of adults are cured. Treatment of t(9;22) positive ALLs with BCR-ABL kinase inhibitors in combination with conventional chemotherapy is highly effective and has greatly improved the outcome in children. The outlook for adults with ALL remains more guarded, in part because of differences

B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and classical Hodgkin

High-grade B-cell lymphoma, with MYC and BCL2 and/or BCL6 rearrangements\*

**Figure 1.** *B-lymphoblastic lymphoma showing CD20, Tdt positivity.*

in the molecular pathogenesis of adult and childhood ALL, but also because older adults cannot tolerate the very intensive chemotherapy regimens that are curative in children. Factors associated with worse prognosis includes: (1) age younger than 2 years, (2) presentation in adolescence or adulthood; and (3) peripheral blood blast counts greater than 100,000. Factors associated with favorable prognosis include (1) age between 2 and 10 years, (2) a low white cell count, (3) hyperdiploidy, (4) trisomy of chromosomes 4, 7, and 10, and (5) the presence of a t(12;21) [1, 25, 38].
