HematologyOutlines

Also known as ALL is an Acute Leukemia of lymphoid origin (B-cell origin is called B-ALL and T-cell origin is called T-ALL). Usually the peripheral blood, tissue and/or bone marrow shows an increase in number of lymphoblasts (typically greater than 20% in the peripheral blood &/or bone marrow as seen in many cases of B-ALL).

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Acute Leukemia of myeloid origin. Peripheral blood, tissue and/or bone marrow show increased number of myeloblasts (typically greater than 20% in the peripheral blood &/or bone marrow). Based on WHO-2008 criteria AMLs are divided into 1) AMLs with recurrent cytogenetic abnormalities (which typically have a better prognosis than the other AMLs) such as APL with t(15;17), AML with t(8;21), AML with t(16;16) or inv (16). 2) AML with Myelodysplasia-related changes (usually have a poor prognosis). 3) Therapy-Related AMLs (usually have a poor prognosis) and 4) AML-NOS which can be minimally differentiated, granulocytic origin (e.g. AML-M2), monocytic or myelomonocytic origin (AML-M4 & M5), erythroid origin (AML-M6) , or megakaryocytic origin (AML-M7). The prognosis on AML-NOS group is variable. Note: A blast with Auer rod(s) is by definition a myeloblast. Myeloblasts with auer rod(s) have been noted in some AML-NOS (e.g. M2) and many times noted in APL.

A systemic mature T-cell neoplasm that is associated with HTLV1 virus infection. Hence, this Leukemia/Lymphoma is more common in certain endemic areas such as parts of Japan, Africa and Caribbean islands.

Also known as anaplastic large cell Lymphoma is usually an aggressive mature/maturing T-cell lymphoma that typically involve nodal tissue (e.g. lymph nodes) and sometimes skin (Cutaneous ALCL has better prognosis). The nodal ALCLs can be either ALK (Anaplastic Large cell Kinase) positive (better prognosis and a potential source of therapy) or ALK negative (worse prognosis in nodal ALCLs). Please note that the cutaneous ALCL is typically ALK negative with best prognosis. In Summary: Amongst ALCLs, ALK negative cutaneous ALCL has the best prognosis while the Nodal ALK negative ALCL has the worst prognosis.

Acute Myeloid Leukemia (AML) is an acute leukemia of myeloid origin. Peripheral blood, tissue and/or bone marrow show increased number of myeloblasts (typically greater than 20% in the peripheral blood &/or bone marrow). Based on WHO-2008 criteria AMLs are divided into 1) AMLs with recurrent cytogenetic abnormalities (which typically have a better prognosis than the other AMLs) such as APL with t(15;17), AML with t(8;21), AML with t(16;16) or inv (16). 2) AML with Myelodysplasia-related changes (usually have a poor prognosis). 3) Therapy-Related AMLs (usually have a poor prognosis) and 4) AML-NOS which can be minimally differentiated, granulocytic origin (e.g. AML-M2), monocytic or myelomonocytic origin (AML-M4 & M5), erythroid origin (AML-M6) , or megakaryocytic origin (AML-M7). The prognosis on AML-NOS group is variable. Note: A blast with Auer rod(s) is by definition a myeloblast. Myeloblasts with auer rod(s) have been noted in some AML-NOS (e.g. M2) and many times noted in APL.

Also known as ALCL is usually an aggressive mature/maturing T-cell Lymphoma that typically involve nodal tissue (e.g. lymph nodes) and sometimes skin (Cutaneous ALCL has better prognosis). The nodal ALCLs can be either ALK (Anaplastic Large cell Kinase) positive (better prognosis and a potential source of therapy) or ALK negative (worse prognosis in nodal ALCLs). Please note that the cutaneous ALCL is typically ALK negative with best prognosis. In Summary: Amongst ALCLs, ALK negative cutaneous ALCL has the best prognosis while the Nodal ALK negative ALCL has the worst prognosis.

These are usually aggressive mature/maturing T-cell lymphomas that typically involve nodal tissue (lymph nodes). Patients with this Lymphoma may have an underlying immune dysfunction and many are secondarily associated with EBV virus.

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Acute Promyelocytic Leukemia. Usually has the following translocation, t(15;17) and is responsive to ATRA therapy.

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Also known as B-Lymphoblastic Leukemia/Lymphoma (or B-Acute lymphoblastic leukemia) is an Acute leukemia of lymphoid origin (specifically B-cell origin). Usually the peripheral blood, tissue and/or bone marrow shows an increase in number of lymphoblasts (typically greater than 20% in the peripheral blood &/or bone marrow as seen in many cases of B-ALL).

An aggressive mature/maturing B-cell Lymphoma which is characterized by intermediate-sized cells with a very high mitotic index (ki67 close to %100). The typical immunophenotype is CD19+, CD20+, CD10+, CD5-, and BCL2-. The classic translocation associated with this lymphoma is the t(8;14) which involves c-MYC and IgH (see lymphoma mnemonic diagram below for an easy way to remember this and other common B-cell lymphomas).

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Also known as CLL. An indolent B-cell Leukemia more common in elderly population. Characteristic immunophenotype (dimCD20+, CD19+, CD5+, CD23+, CD10- monotypic dim kappa or dim lambda B-cells). The disease has two forms: CLL if it's in the blood (hence, "Leukemia") and SLL (Small Lymphocytic Lymphoma) if it's in the tissue such as lymph nodes (hence, "Lymphoma"). Mnemonic: As opposed to Acute Lymphoblastic Leukemia (ALL) which are "immature" cells and more common in "pediatric" (less mature) population, CLL is comprised of "mature" lymphocytes and predominantly seen in elderly (more mature) population.

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A Myeloproliferative neoplasm of abnormal bone marrow Stem cells that is characterized by the chromosome translocation t(9;22) which leads to a BCR-ABL1 fusion gene (Philadelphia chromosome). Initially presents as an indolent Chronic phase with subsequent transition to accelerated phase and sometimes blast phase. First line therapy usually includes "imatinib" (AKA Gleevec). Similar to most of the other myeloproliferative neoplasms, patients usually present with some elevated count or "cytosis" (such as elevated WBC count which is known as leukocytosis) and splenomegaly. The peripheral blood smear usually shows leukocytosis with increased number of myeloid (granulocytic) precursors in different phases of maturation (Clue: the peripheral blood smear looks like a bone marrow aspirate smear).

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AKA CMML is an abnormal bone marrow Stem cell disorder that has characteristic features of both a Myeloproliferative neoplasm ("cytosis" specifically Monocytosis and splenomegaly) and a myelodysplastic syndrome (dysplastic cells). Hence, per WHO 2008 criteria, it belongs to the category of Myeloproliferative/Myelodysplastic disorders. At the time of diagnosis, they usually present with leukocytosis and history of a "persistent Monocytosis" is typically required. As opposed to CML, there is no BCR-ABL1 fusion.

Also known as Chronic Lymphocytic Leukemia. An indolent B-cell leukemia more common in elderly population. Characteristic immunophenotype (dimCD20+, CD19+, CD5+, CD23+, CD10- monotypic dim kappa or dim lambda B-cells). The disease has two forms: CLL if it's in the blood (hence, "Leukemia") and SLL (Small Lymphocytic Lymphoma) if it's in the tissue such as lymph nodes (hence, "Lymphoma"). Mnemonic: As opposed to Acute Lymphoblastic Leukemia (ALL) which are "immature" cells and more common in "pediatric" (less mature) population, CLL is comprised of "mature" lymphocytes and predominantly seen in elderly (more mature) population.

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Chronic Myelogenous Leukemia. A Myeloproliferative neoplasm of abnormal bone marrow Stem cells that is characterized by t(9;22) which leads to a BCR-ABL1 fusion gene (Philadelphia chromosome). Initially presents as an indolent chronic phase with subsequent transition to accelerated phase and sometimes blast phase. First line therapy usually includes "imatinib" (AKA Gleevec). Similar to most of the other myeloproliferative neoplasms, patients usually present with some "cytosis" (such as elevated WBC which is known as leukocytosis) and splenomegaly. The peripheral blood smear usually shows leukocytosis with increased number of myeloid (granulocytic) precursors in different phases of maturation (Clue: the peripheral blood smear looks like a bone marrow aspirate smear).

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AKA Chronic MyeloMonocytic Leukemia is an abnormal bone marrow Stem cell disorder that has characteristic features of both a Myeloproliferative neoplasm ("cytosis" specifically Monocytosis and splenomegaly) and a myelodysplastic syndrome (dysplastic cells). Hence, per WHO 2008 criteria, it belongs to the category of Myeloproliferative/Myelodysplastic disorders. At the time of diagnosis, they usually present with leukocytosis and history of a "persistent Monocytosis" is typically required. As opposed to CML, there is no BCR-ABL1 fusion.

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A common mature B-cell Lymphoma that usually shows a nodular growth pattern and more commonly behaves in a low grade fashion. Immunophenotypically the neoplastic B-cells in this lymphoma are CD19+ and CD20+ ("Big" CDs meaning "B" cell origin), CD10+ and BCL2+. The common translocation associated with this lymphoma is t(14;18) which includes IgH and BCL2 genes, see lymphoma_mnemonic.

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An indolent B-cell Leukemia with leukemic cells (abnormal cells circulating in the blood) that have cytoplasmic projections resembling "Hair-like" projections. Patients usually present with some cytopenia and splenomegaly with the neoplastic cells involving the red pulp of the spleen. The typical immunophenotype of hairy cell is CD19+, CD20+, CD25+, CD103+ and CD11c+.

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An indolent B-cell Lymphoma that usually presents in supradiaphragmatic areas (above the diaphragm such as supraclavicular lymph nodes or Anterior Mediastinum). There is a bimodal age distribution (most patients present in their 20s and 50s). Four main subtypes are: Nodular Sclerosis, Mixed Cellularity, Lymphocyte-rich, and Lymphocyte-depleted. The hallmark of all Classical Hodgkin lymphomas is the Reed-Sternberg cell (RS cell) which usually have the following immunophenotype: dim PAX5+, CD30+, CD15+, CD20-, OCT2- and BOB1-.

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Includes Diffuse Large B-cell Lymphoma (DLBCL) and anaplastic large cell lymphoma (usually a T-cell lymphoma). By definition the neoplastic cells in these lymphomas are typically three times the size or larger than small mature lymphocytes.

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A mnemonic for common translocations associated with several mature B-cell lymphomas.

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An indolent B-Lymphoma which is characterized by small-intermediate sized neoplastic lymphocytes and plasma cells. Often associated with Waldenstrom Macrogammaglobulinemia (usually showing an increased amount of clonal IgM by serum protein electrophoresis and Immunofixation Electrophoresis).

An intermediately (between a low grade and high grade Lymphoma) aggressive CD5 positive CD23 negative B-cell lymphoma that is usually due to t(11;14) leading to the overexpression of Cyclin D1 (BCL1). This is in contrast to CLL which is usually CD5+,CD23+ and BCL1 (CyclinD1) negative.

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A group of mature or maturing myeloid neoplasms that usually share the following features: Cytosis refers to the increase in various blood counts (red count, white count, platelet count) with the exception of Primary Myelofibrosis), splenomagly (less common in ET patients), and hypercellular marrow (except in Primary Myelofibrosis or spent phase of the some MPNs). The most common MPNs include CML, PV, ET, and Primary Myelofibrosis. CML has the t(9;22) translocation (BCR/ABL1) while PV, ET and Primary Myelofibrosis usually show the JAK2 mutation.

Also known as plasma cell Myeloma. A plasma cell neoplasm that is usually characterized by increased numbers (10% or more) of monoclonal plasma cells in the bone marrow. Two main types: Asymptomatic (Smoldering) and Symptomatic (characterized by one or more of the following CRAB findings: Remember "CRAB", C for hyperCalcemia, R for Renal disease associated with multiple myeloma, A for anemia associated with the multiple myeloma, and B for lytic Bone lesions).

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Also known as MF is the most common skin lymphoproliferative disorder. These are usually CD4+ T-cell lymphomas with characteristic epidermotropism (neoplastic cells crawling up into the epidermis) and Pautrier's micro abscesses (collection of neoplastic cells around a dendritic cell in the epidermis)

Also known as MDS is a heterogeneous group of Chronic myeloid neoplasms which usually share the following features: cytopenia (decrease in number of a cell line in blood with the exception of MDS with isolated 5q- which may show an increase in platelets), lack of splenomegaly and a Hypercellular marrow. The most common MDS include RAEB-I, RAEB-II, RCUD (e.g. RA or RARS), RCMD, and MDS with isolated 5q-. RAEB have the worst prognosis (higher liklihod of transforming into AML) while MDS with isolated 5q- has the best prognosis.

A group of mature or maturing myeloid neoplasms that usually share the following features: Cytosis (increase in a cell line in blood with the exception of Primary Myelofibrosis), Splenomagly (less common in ET patients), and Hypercellular marrow (except in Primary Myelofibrosis or spent phase of the some MPNs). The most common MPNs include CML, PV, ET, and Primary Myelofibrosis. CML has the t(9;22) translocation (BCR/ABL1) while PV, ET and Primary Myelofibrosis many show the JAK2 mutation.

These are mature/maturing neoplastic lymphoid cells (Lymphoma) of either B-cell, NK-cell or T-cell lineage. Examples include Diffuse Large B-Cell Lymphoma (DLBCL) and follicular_lymphoma which are of B-cell origin while Anaplastic Large Cell Lymhphoma (ALCL) and Peripheral T-Cell Lymphoma Not Otherwise Specified (PTCL-NOS) are of T-cell origin.

This is a very aggressive plasma cell neoplasm with an increased number circulating plasma cells in the peripheral blood (usually >20% of WBCs).

Also known as multiple myeloma is a plasma cell neoplasm that is usually characterized by increased numbers (10% or more) of monoclonal plasma cells in the bone marrow. Two main types: Asymptomatic (Smoldering) and Symptomatic (characterized by one or more of the following CRAB findings: Remember "CRAB", C for hyperCalcemia, R for Renal disease associated with multiple myeloma, A for anemia associated with the multiple myeloma, and B for lytic Bone lesions).

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A plasma cell neoplasm that consists almost entirely of neoplastic plasma cells involving tissue outside of the bone marrow.

A myeloprolifeartive neoplasm that usually carries the JAK2 mutation. Patients with this neoplasm typically present with splenomegaly, and some "cytosis" which is usually in the form of an elevated hematrocrit/hemoglobin with low EPO levels. The bone marrow is usually hypercellular similar to many of the other myeloproliferative neoplasms.

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Small Lymphocytic Lymphoma is an indolent B-cell lymphoma and in simple terms Chronic lymphocytic Leukemia (CLL) involving tissue such as lymph nodes (hence the term lymphoma) . The characteristic immunophenotype is similar to CLL showing dim CD20 (+), CD19 (+), CD5 (+), CD23 (+), CD10 (-), and monotypic dim surface kappa or lambda light chain restricted neoplastic B-cells.

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Also known as SLL is an indolent B-cell Lymphoma and in simple terms Chronic lymphocytic Leukemia (CLL) involving tissue such as lymph nodes (hence the term lymphoma) . The characteristic immunophenotype is similar to CLL showing dim CD20 (+), CD19 (+), CD5 (+), CD23 (+), CD10 (-), and monotypic dim surface kappa or lambda light chain restricted neoplastic B-cells.

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A low-grade mature B-cell Lymphoma that usually involves the spleen, peripheral blood and sometimes other organs such as the bone marrow. Characteristic features are splenomegaly (involving the white pulp of spleen as opposed to hairy cell Leukemia which involves the splenic red pulp), moderate lymphocytosis with villous morphology (seen in the peripheral blood smear), and intrasinusoidal pattern of involvement of various organs, especially the bone marrow. Characteristic immunophenotype is similar to other marginal zone lymphomas (triple negative phenotype referring to being negative for CD5, CD10 and CD23).

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Also known as T-Lymphoblastic Leukemia/Lymphoma (or T-Acute lymphoblastic leukemia) is an Acute leukemia of lymphoid origin (specifically T-cell origin). Many involve the anterior mediastinum (thymus). Recall the mnemonic of 4 "Ts" for Anterior Mediastinal Lesions: T for Terrible lymphomas (e.g. T-ALL, although Hodgkin lymphoma many times may involve anterior mediastinum and is not as aggressive as T-ALL), T for Thymoma, T for Thyroid remnant, and T for Teratoma (or other germ cell neoplasms).

T-cell lymphomas are a group of Non-Hodgkin lymphomas (NHLs) that are the result of proliferating neoplastic T-lymphocytes (T-cells). They account for approximately 15% of the NHL group. Some of the more common types of T-cell lymphomas include Peripheral T-cell Lymphoma NOS, angioimmunoblastic t-cell lymphoma, Cutaneous T-cell lymphomas (most are Mycoises Fungoides), anaplastic large cell lymphoma (ALCL), T-LGL Leukemia and Adult T-cell lymphoma/leukemia (ATLL).

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Neoplasms