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pluripotent hematopoietic stem cells in either the bone marrow or lymphoid tissue

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Leukocytes originate from

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the same stem cell precursors in the bone marrow

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Granulocytes and monocytes are derived from

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lymph nodes

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lymphocytes originate in the

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immature precursors

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mitotic pool consists of

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of white blood cells (WBC's) undergoing maturation

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the maturing pool consist

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functional WBC's that can be released when needed

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the storage pool consists of

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neutrophils, eosinophils, and basophils

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The three types of granulocytes are

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Neutrophils

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most dominant of all circulating phagocytes

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Neutrophils

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They provide the first line of defense against bacterial invasion of the mucous membranes and skin, and comprise greater than half of all leukocytes

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must respond to chemotactic stimuli and migrate to the site of tissue damage, must be capable of phagocytosis, they must destroy bacteria through enzymatic activity

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Three cellular functions must be intact for neutrophils to provide this protection against infection - what are they?

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the bone marrow

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The largest number of neutrophils (90%) can be found in

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circulating and marginal

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neutrophils are released into the circulation into two pools

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Basophils

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migrate to tissues carrying heparin and histamine- and platelet-activating factors, and they act as mast cells in allergic reactions

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Lymphocytes

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primary cells involved in immunity

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Quantitative disorders

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result from an abnormal number of white cells

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Qualitative disorders

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result from abnormal / poorly functioning cells

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Myeloproliferative disease

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results from acquired clonal abnormalities of hematopoietic stem cells

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leukemias

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are considered to be myeloproliferative diseases

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Malignancies

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the most important disorders of white cells

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Myeloid neoplasms, Lymphoid neoplasms

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Malignancies fall into several broad categories

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Acute myeloid leukemias, Myelodysplastic syndromes, Chronic myeloproliferative disorders

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Myeloid neoplasms arise from early hematopoietic progenitors. Three categories of myeloid neoplasia are recognized

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Acute myeloid leukemias

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in which immature progenitor cells accumulate in the bone marrow

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Myelodysplastic syndromes

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are associated with ineffective hematopoiesis and resultant peripheral blood cytopenias

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Chronic myeloproliferative disorders

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increased production of one or more terminally differentiated myeloid elements (e.g., granulocytes, erythrocytes) usually leads to elevated peripheral blood counts

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Chronic myelogenous leukemia, Polycythemia vera (PV), Essential thrombocytosis (primary thrombocythemia), Primary myelofibrosis, Histiocytosis

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Examples of Chronic myeloproliferative disorders

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Polycythemia vera (Hepatomegaly is common, and more than 75% of patients have splenomegaly (which may be massive))

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most prominent feature of this disease is an elevated absolute red blood cell mass due to uncontrolled red blood cell production, which increases hematocrit and blood viscosity and may lead to thrombosis

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Primary myelofibrosis

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characterized by the development of obliterative marrow fibrosis

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Histiocytosis

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the accumulation and infiltration of monocytes, macrophages, and dendritic cells in the affected tissues

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Histiocytosis

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A special type of immature dendritic cell, the Langerhans cell, gives rise to a spectrum of neoplastic disorders referred to as the Langerhans cell histiocytosis and is associated with

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Lymphoid neoplasms

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diverse group of tumors of B-cell, T-cell, and NK-cell origin

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Acute leukemia (cells proliferate in an uncontrolled fashion and replace normal bone marrow elements)

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is a malignancy of the hematopoietic progenitor cell

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Acute myelogenous leukemia (AML)

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is a disorder characterized by uncontrolled proliferation of primitive myeloid cells (blasts)

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adults (most patients are 30 to 60 years old; median age at presentation is 50 years) Annual incidence is 2 to 4 per 100,000 persons

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AML usually affects

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Acute lymphoblastic leukemia (ALL) or Lymphoid leukemia

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characterized by uncontrolled proliferation of abnormal, immature lymphocytes and their progenitors, ultimately replacing normal bone marrow elements

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children (peak incidence at ages 2 to 10 years) and comprises 80% of the acute leukemias of childhood, 3000 to 4000 persons in the U.S. each year; two-thirds are children

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ALL is primarily a disease of

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antineoplastic agents, chromosomal abnormalities, ionizing radiation, toxins, congenital genetic disorders/syndromes, and chronic myeloproliferative disorders

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Known risk factors for acute leukemia include

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leukemogenic

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Radiation exposure is

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benzene and toluene

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Toxins that are known causes of acute leukemia

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chromosomes 5 and 7

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leukemias seen after toxin or chemotherapy exposure often develop from a myelodysplastic prodrome and are associated with abnormalities in

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may cause leukemia

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What is important to know about antineoplastic chemotherapeutic agents

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gain-of-function mutations in NOTCH1, a gene that is essential for T-cell development

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Up to 70% of T-ALLs have what kind of mutations

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chronic myelogenous leukemia

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Philadelphia chromosome (Ph) is associated with

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[t(9;22)], a translocation that results in fusion of the bcr gene on chromosome 22 to the abl tyrosine kinase gene on chromosome 9

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What is Philadelphia chromosome (Ph)

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chromosomal translocation t(15;17), which produces the fusion gene PML-RARa which interacts with the retinoic acid receptor to produce a block in cell differentiation and can be treated with retinoic acid

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Acute promyelocytic leukemia (APL) (a uncommon sub-type of AML), is characterized by

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mutation at 21q22

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Rare cases of familial erythroleukemia (a subtype of AML), have been described due to

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of an AHD, the most common of which is myelodysplastic syndrome (MDS)

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The most common risk factor for AML is the presence

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Antecedent Hematologic Disorders (AHD)

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What is AHD

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primary (idiopathic) or secondary to previous genotoxic drug or radiation therapy (t-MDS)

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MDS may be either

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AML

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All forms of MDS can have a significant risk to transform to

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Primary MDS

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predominantly a disease of the elderly; the mean age of onset is 70 years

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progressive cytopenias that occur over months to years

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Primary MDS manifests as

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aplastic anemia, myelofibrosis, paroxysmal nocturnal hemoglobinuria, and polycythemia vera

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Other AHDs that predispose patients to AML include

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blast phase (or blast crisis) that is indistinguishable from AML

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most patients with chronic myelogenous leukemia (a myeloproliferative disorder), eventually develop a

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Bloom syndrome (congenital telangiectatic erythema), *Down syndrome (has a 20-fold increase in the risk for acute leukemia), congenital neutropenia, Fanconi anemia, and neurofibromatosis

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Some congenital disorders predispose patients to AML and they include

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either bone marrow suppression or infiltration of leukemic cells into other organs and tissues (anemia, thrombocytopenia, and a decrease in normally functioning neutrophils)

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symptoms and signs of acute leukemia result from

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pallor, shortness of breath, and fatigue, which is the most common presenting symptom

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In acute leukemia the anemia results in

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spontaneous bleeding, such as petechiae, ecchymoses, epistaxis, melena, increased menstrual bleeding, and gingival bleeding

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Thrombocytopenia causes

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disseminated intravascular coagulation (DIC)

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can result from substances released from leukemic cells that activate coagulation

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ironic combination of thrombosis and hemorrhage owing to depletion of coagulation factors

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DIC patients, who usually have promyelocytic leukemia, have the

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Infection (death within a few hours may occur if treatment with appropriate antibiotics is delayed)

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is therefore a frequent complication of the disease (acute leukemia) and is the most common cause of morbidity and mortality

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gram-negative bacteria (Escherichia coli, Klebsiella, Pseudomonas) or fungi (Candida, Aspergillus)

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The most common pathogens (involved in infection of those with acute leukemia) are

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Fever

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is an early sign of disease owing to recurrent infections of the lungs, urinary tract, skin, mouth, rectum, and upper respiratory tract

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lymphadenopathy, hepatomegaly, and splenomegaly

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Infiltration of organs and tissues by leukemic cells causes

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Stomatitis and gingival swelling (infiltrate)

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may be seen in patients with leukemia, especially monocytic (monoblastic) leukemia

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central nervous system or peripheral nerves, leading to cranial nerve palsy, paresthesia, anesthesia, and paralysis

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Leukemic cells may infiltrate the

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"chloromas" or "myeloblastomas". The surface of these tumors turns **green when exposed to light because of the presence of myeloperoxidase

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Localized tumors consisting of leukemic cells are called

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laboratory examination of the peripheral blood and bone marrow

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The diagnosis of acute leukemia is made with

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Pancytopenia with circulating blasts

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is usually considered to be pathognomonic for acute leukemia, however, this is not seen in many cases

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More than 20-30% (abnormal) blasts in the bone marrow

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usually required to make a diagnosis of acute leukemia

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high incidence of CNS involvement

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A lumbar puncture is indicated in patients with ALL because of the

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Auer rod

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eosinophilic needle-like inclusion in the cytoplasm, is pathognomonic of AML and is called what

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French-American-British (FAB) classification

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AML is usually categorized using the

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ALL

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This is considered when there is no morphologic or histochemical evidence of myeloid or monocytic lineage

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CD19, common to all B cells, and most cases will express CD10

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ALL cells of B lineage will express

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terminal deoxynucleotidyl transferase (TdT)

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Almost all (~ 95%) ALL cells express

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remission

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The first step in treatment is to obtain complete

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chemotherapy

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Most acute leukemias are initially treated with

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Induction, Consolidation, and Maintenance

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Chemotherapy is divided into three stages

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It usually takes 28 to 32 days from the start of therapy to achieve remission

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How long does it take to achieve remission

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1 year

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median duration of remission in an adult with AML is

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chemotherapy with or without radiation and its purpose is to prolong remission

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Consolidation therapy consists of an aggressive course of

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severe bone marrow suppression (anemia, thrombocytopenia, granulocytopenia)

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Complications of consolidation therapy are usually attributable to

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central nervous system prophylaxis

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After achieving complete remission, patients receive this so that meningeal sequestration of leukemic cells does not develop

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less than 55 years old without a HLA-matched sibling donor

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Autologous bone marrow transplantation or hemopoietic stem cell transplant (HSCT) is indicated in patients

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Allogeneic bone marrow transplantation (potential cure)

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generally available to less than 20% of patients

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Hemopoietic stem cell transplant (HSCT)

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is an advancement over bone marrow transplantation (BMT)

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peripheral blood, bone marrow, umbilical cord blood, and, rarely, fetal liver

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Hematopoietic stem cells can be collected for transplant use from several sources, including

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syngeneic

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from a genetically identical twin

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allogeneic

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from a donor who is genetically similar but not identical

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autologous

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a portion of the patient's own stem cells (or bone marrow) is removed prior to chemotherapy, screened [to eliminate malignant cells], preserved, and re-implanted after therapy

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high-dose (ablative) cytotoxic chemotherapy and/or total body irradiation (TBI) in which the patient's bone marrow cells are destroyed (myeloablation) = susceptible to infection

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Myeloablative HSCT is preceded by a combination of

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acute and chronic graft-versus-host disease (GVHD) caused by T-lymphocytes from the graft that destroy normal vital host tissues and organs

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After engraftment, complications include

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Nonmyeloablative (also referred to as reduced-intensity conditioning [RIC]) allogeneic and autologous HSCT

answer

are in many cases potentially as effective as conventional myeloablative allogeneic HSCTs

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Hodgkin disease, multiple myeloma, and low-grade lymphoid malignancies

answer

diseases most impacted by RIC regimens

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allogenic RIC HSCT

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donor stem cells (usually from an HLA-matched sibling) are infused into the patient

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mixed chimerism

answer

patient has a mix of his native immune system and the donor immune system

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full chimerism

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new (donor) immune system will gradually take over until no trace of the patient's original immune system remains

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induction, consolidation, maintenance, and CNS prophylaxis

answer

Traditionally, the 4 components of ALL chemotherapy treatment are

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daunorubicin, vincristine, prednisone, and L- asparaginase given over the course of 4 to 6 weeks

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Standard induction therapy in adults with ALL typically involves combination chemotherapy, including

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tyrosine kinase inhibitor added to initial chemotherapy

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patients with Philadelphia chromosome-positive ALL should have a

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Maintenance therapy

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Intermittent therapy for at least 3 years

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meningeal leukemia at the time of relapse making CNS prophylaxis essential

answer

In contrast to patients with AML, patients with ALL frequently have

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