Types of Myelodysplastic Syndromes (MDS) There Are Many Subtypes Of

Total Page:16

File Type:pdf, Size:1020Kb

Types of Myelodysplastic Syndromes (MDS) There Are Many Subtypes Of Types of Myelodysplastic Syndromes (MDS) There are many subtypes of Myelodysplastic Syndromes (MDS). For each patient, the subtype is determined using the results of the blood and bone marrow tests your MDS physician will perform. There are many classification systems. Generally MDS can be divided into the following subtypes: • Refractory anemia. There are too few red blood cells in the blood and the patient has anemia. The number of white blood cells and platelets is normal. • Refractory anemia with ringed sideroblasts. There are too few red blood cells in the blood and the patient has anemia. The red blood cells have too much iron. The number of white blood cells and platelets is normal. • Refractory anemia with excess blasts. There are too few red blood cells in the blood and the patient has anemia. Five percent to 19% of the cells in the bone marrow are blasts and there are a normal number of blasts found in the blood. There also may be changes to the white blood cells and platelets. Refractory anemia with excess blasts has a higher risk of progressing to acute myeloid leukemia. • Refractory cytopenia with multilineage dysplasia. There are too few of at least two types of blood cells. Less than 5% of the cells in the bone marrow are blasts and less than 1% of the cells in the blood are blasts. If red blood cells are affected, they may have extra iron. Refractory cytopenia may progress to acute leukemia. • Myelodysplastic syndrome associated with an isolated del(5q) chromosome abnormality. There are too few red blood cells in the blood and the patient has anemia. Less than 5% of the cells in the bone marrow and blood are blasts. There is a specific change in the chromosome. • Unclassifiable myelodysplastic syndrome. There are too few of one type of blood cell in the blood. The number of blasts in the bone marrow and blood is normal, and the disease is not one of the other myelodysplastic syndromes. • Chronic Myelomonocytic Leukemia (CMMoL). Excessive numbers of monocytes (a type of white blood cell) are seen in the marrow and blood. 5 to 20 percent blasts in the bone marrow; less than 5 percent blasts in the blood. Cytopenia of at least two cell lines. .
Recommended publications
  • The Clinical Management of Chronic Myelomonocytic Leukemia Eric Padron, MD, Rami Komrokji, and Alan F
    The Clinical Management of Chronic Myelomonocytic Leukemia Eric Padron, MD, Rami Komrokji, and Alan F. List, MD Dr Padron is an assistant member, Dr Abstract: Chronic myelomonocytic leukemia (CMML) is an Komrokji is an associate member, and Dr aggressive malignancy characterized by peripheral monocytosis List is a senior member in the Department and ineffective hematopoiesis. It has been historically classified of Malignant Hematology at the H. Lee as a subtype of the myelodysplastic syndromes (MDSs) but was Moffitt Cancer Center & Research Institute in Tampa, Florida. recently demonstrated to be a distinct entity with a distinct natu- ral history. Nonetheless, clinical practice guidelines for CMML Address correspondence to: have been inferred from studies designed for MDSs. It is impera- Eric Padron, MD tive that clinicians understand which elements of MDS clinical Assistant Member practice are translatable to CMML, including which evidence has Malignant Hematology been generated from CMML-specific studies and which has not. H. Lee Moffitt Cancer Center & Research Institute This allows for an evidence-based approach to the treatment of 12902 Magnolia Drive CMML and identifies knowledge gaps in need of further study in Tampa, Florida 33612 a disease-specific manner. This review discusses the diagnosis, E-mail: [email protected] prognosis, and treatment of CMML, with the task of divorcing aspects of MDS practice that have not been demonstrated to be applicable to CMML and merging those that have been shown to be clinically similar. Introduction Chronic myelomonocytic leukemia (CMML) is a clonal hemato- logic malignancy characterized by absolute peripheral monocytosis, ineffective hematopoiesis, and an increased risk of transformation to acute myeloid leukemia.
    [Show full text]
  • Bone Marrow (Stem Cell) Transplant for Sickle Cell Disease Bone Marrow (Stem Cell) Transplant
    Bone Marrow (Stem Cell) Transplant for Sickle Cell Disease Bone Marrow (Stem Cell) Transplant for Sickle Cell Disease 1 Produced by St. Jude Children’s Research Hospital Departments of Hematology, Patient Education, and Biomedical Communications. Funds were provided by St. Jude Children’s Research Hospital, ALSAC, and a grant from the Plough Foundation. This document is not intended to take the place of the care and attention of your personal physician. Our goal is to promote active participation in your care and treatment by providing information and education. Questions about individual health concerns or specifi c treatment options should be discussed with your physician. For more general information on sickle cell disease, please visit our Web site at www.stjude.org/sicklecell. Copyright © 2009 St. Jude Children’s Research Hospital How did bone marrow (stem cell) transplants begin for children with sickle cell disease? Bone marrow (stem cell) transplants have been used for the treatment and cure of a variety of cancers, immune system diseases, and blood diseases for many years. Doctors in the United States and other countries have developed studies to treat children who have severe sickle cell disease with bone marrow (stem cell) transplants. How does a bone marrow (stem cell) transplant work? 2 In a person with sickle cell disease, the bone marrow produces red blood cells that contain hemoglobin S. This leads to the complications of sickle cell disease. • To prepare for a bone marrow (stem cell) transplant, strong medicines, called chemotherapy, are used to weaken or destroy the patient’s own bone marrow, stem cells, and infection fi ghting system.
    [Show full text]
  • Outcomes for Patients with Chronic Lymphocytic Leukemia and Acute Leukemia Or Myelodysplastic Syndrome
    Leukemia (2016) 30, 325–330 © 2016 Macmillan Publishers Limited All rights reserved 0887-6924/16 www.nature.com/leu ORIGINAL ARTICLE Outcomes for patients with chronic lymphocytic leukemia and acute leukemia or myelodysplastic syndrome FP Tambaro1, G Garcia-Manero2, SM O'Brien2, SH Faderl3, A Ferrajoli2, JA Burger2, S Pierce2, X Wang4, K-A Do4, HM Kantarjian2, MJ Keating2 and WG Wierda2 Acute leukemia (AL) and myelodysplastic syndrome (MDS) are uncommon in chronic lymphocytic leukemia (CLL). We retrospectively identified 95 patients with CLL, also diagnosed with AL (n = 38) or MDS (n = 57), either concurrently (n =5)or subsequent (n = 90) to CLL diagnosis and report their outcomes. Median number of CLL treatments prior to AL and MDS was 2 (0–9) and 1 (0–8), respectively; the most common regimen was purine analog combined with alkylating agent±CD20 monoclonal antibody. Twelve cases had no prior CLL treatment. Among 38 cases with AL, 33 had acute myelogenous leukemia (AML), 3 had acute lymphoid leukemia (ALL; 1 Philadelphia chromosome positive), 1 had biphenotypic and 1 had extramedullary (bladder) AML. Unfavorable AML karyotype was noted in 26, and intermediate risk in 7 patients. There was no association between survival from AL and number of prior CLL regimens or karyotype. Expression of CD7 on blasts was associated with shorter survival. Among MDS cases, all International Prognostic Scoring System (IPSS) were represented; karyotype was unfavorable in 36, intermediate in 6 and favorable in 12 patients; 10 experienced transformation to AML. Shorter survival from MDS correlated with higher risk IPSS, poor-risk karyotype and increased number of prior CLL treatments.
    [Show full text]
  • Myelodysplastic Syndromes Overview and Types
    cancer.org | 1.800.227.2345 About Myelodysplastic Syndromes Overview and Types If you have been diagnosed with a myelodysplastic syndrome or are worried about it, you likely have a lot of questions. Learning some basics is a good place to start. ● What Are Myelodysplastic Syndromes? ● Types of Myelodysplastic Syndromes Research and Statistics See the latest estimates for new cases of myelodysplastic syndromes in the US and what research is currently being done. ● Key Statistics for Myelodysplastic Syndromes ● What's New in Myelodysplastic Syndrome Research? What Are Myelodysplastic Syndromes? Myelodysplastic syndromes (MDS) are conditions that can occur when the blood- forming cells in the bone marrow become abnormal. This leads to low numbers of one or more types of blood cells. MDS is considered a type of cancer1. Normal bone marrow 1 ____________________________________________________________________________________American Cancer Society cancer.org | 1.800.227.2345 Bone marrow is found in the middle of certain bones. It is made up of blood-forming cells, fat cells, and supporting tissues. A small fraction of the blood-forming cells are blood stem cells. Stem cells are needed to make new blood cells. There are 3 main types of blood cells: red blood cells, white blood cells, and platelets. Red blood cells pick up oxygen in the lungs and carry it to the rest of the body. These cells also bring carbon dioxide back to the lungs. Having too few red blood cells is called anemia. It can make a person feel tired and weak and look pale. Severe anemia can cause shortness of breath. White blood cells (also known as leukocytes) are important in defending the body against infection.
    [Show full text]
  • Your Blood Cells
    Page 1 of 2 Original Date The Johns Hopkins Hospital Patient Information 12/00 Oncology ReviseD/ RevieweD 6/14 Your Blood Cells Where are Blood cells are made in the bone marrow. The bone marrow blood cells is a liquid that looks like blood. It is found in several places of made? the body, such as your hips, chest bone and the middle part of your arm and leg bones. What types of • The three main types of blood cells are the red blood cells, blood cells do the white blood cells and the platelets. I have? • Red blood cells carry oxygen to all parts of the body. The normal hematocrit (or percentage of red blood cells in the blood) is 41-53%. Anemia means low red blood cells. • White blood cells fight infection. The normal white blood cell count is 4.5-11 (K/cu mm). The most important white blood cell to fight infection is the neutrophil. Forty to seventy percent (40-70%) of your white blood cells should be neutrophils. Neutropenia means your neutrophils are low, or less than 40%. • Platelets help your blood to clot and stop bleeding. The normal platelet count is 150-350 (K/cu mm). Thrombocytopenia means low platelets. How do you Your blood cells are measured by a test called the Complete measure my Blood Count (CBC) or Heme 8/Diff. You may want to keep track blood cells? of your blood counts on the back of this sheet. What When your blood counts are low, you may become anemic, get happens infections and bleed or bruise easier.
    [Show full text]
  • Automatic Blood Cell and CRP Counter with Three-Part Differential
    FEATURE ARTICLE Automatic Blood Cell and CRP Counter with Three-Part Differential Measurement of White Blood Cells The LC-170 CRP FEATURE ARTICLE Automatic Blood Cell and CRP Counter with Three-Part Differential Measurement of White Blood Cells, The LC-170 CRP Yasuo Yamao WBC, RBC, Hct Electrical impedance method Hgb CRP Cyanmethemoglobin method Latex immunoturbidmetry WBC (White blood cells) LYM% MON% GRA% CRP quantitative LC-170CRP (Lymphocyte %) (Monocyte %) (Granulocyte %) analysis LYM# MON# GRA# (C-reactive protein) (Lymphocyte No.) (Monocyte No.) (Granulocyte No.) RBC (Red blood cells) PLT (Platelets) Hgb(Hemoglobin) Pct (Plateletcrit) Hct (Hematocrit) MPV (Mean Platelet Volume) MCV (Mean Corpuscular Volume) PDW (Platelet Distribution Width) MCH (Mean Corpuscular Hemoglobin) MCHC (Mean Corpuscular Hemoglobin Concentration) RDW (Red Blood Cell Distribution Width) Example of results Abstract The LC-l70 CRP automatic blood cell and CRP counter, developed by Horiba, is capable of simultaneously measuring all 19 C-reactive protein (CRP) density parameters and counting red blood cells, platelets, and three types of white blood cell: lymphocytes, monocytes, and granulocytes. As clinicians demand ever-higher precision measurements, a need has developed for clinical test machines having excellent operational and cost performance. This compact machine should make a powerful tool for initial diagnosis of inflammatory and infectious diseases, especially at small- and mid-size medical institutions. 20 Technical Reports 1 Introduction 2 Measurement Principles To prevent an explosion of medical costs as Japanese The LC-170 CRP uses the electrical impedance method society ages and fewer children are born, the Ministry of to count blood cells, the cyanmethemoglobin method to Health, Labor, and Welfare is pursuing a thorough reform measure hemoglobin concentration, and latex of the medical insurance system, including “preventing immunoturbidimetry to measure CRP concentration.
    [Show full text]
  • Essential Thrombocythemia Facts No
    Essential Thrombocythemia Facts No. 12 in a series providing the latest information for patients, caregivers and healthcare professionals www.LLS.org • Information Specialist: 800.955.4572 Introduction Highlights Essential thrombocythemia (ET) is one of several l Essential thrombocythemia (ET) is one of a related “myeloproliferative neoplasms” (MPNs), a group of closely group of blood cancers known as “myeloproliferative related blood cancers that share several features, notably the neoplasms” (MPNs) in which cells in the bone “clonal” overproduction of one or more blood cell lines. marrow that produce the blood cells develop and All clonal disorders begin with one or more changes function abnormally. (mutations) to the DNA in a single cell; the altered cells in l ET begins with one or more acquired changes the marrow and the blood are the offspring of that one (mutations) to the DNA of a single blood-forming mutant cell. Other MPNs include polycythemia vera and cell. This results in the overproduction of blood cells, myelofibrosis. especially platelets, in the bone marrow. The effects of ET result from uncontrolled blood cell l About half of individuals with ET have a mutation production, notably of platelets. Because the disease arises of the JAK2 (Janus kinase 2) gene. The role that this from a change to an early blood-forming cell that has the mutation plays in the development of the disease, capacity to form red cells, white cells and platelets, any and the potential implications for new treatments, combination of these three cell lines may be affected – and are being investigated. usually each cell line is affected to some degree.
    [Show full text]
  • Neutrophils (A.K.A
    Neutrophils (a.k.a. leukocyte, granulocyte, white blood cell, WBC) blood cells on a microscope slide showing the lobed nucleus of two neutrophils a 3-d drawing of a neutrophil This chart shows how blood cells are produced in the bone marrow from “stem cells” and how we organize them into groups. By Mikael Häggström The Mul(faceted Func(ons of Neutrophils Tanya N. Mayadas,1 Xavier Cullere,1 and Clifford A. Lowell2 “Neutrophils and neutrophil-like cells are the major pathogen-fighKng immune cells in organisms ranging from slime molds to mammals. Central to their funcKon is their ability to be recruited to sites of infecKon, to recognize and phagocytose (eang foreign objects like bacteria) microbes, and then to kill pathogens through a combinaon of cytotoxic mechanisms.” Form • average diameter of 12-15 micrometers (µm) in peripheral blood smears • in suspension, neutrophils have an average diameter of 8.85 µm • mulK-lobed shape nucleus • survive for only 8–12 h in the circulaon and up to 1–2 days in Kssues • Neutrophils will show increasing segmentaon (many segments of nucleus) as they mature. A normal neutrophil should have 3-5 segments. • Neutrophils account for approximately 50-70% of all white blood cells (leukocytes) Func(on • first line of host defense against a wide range of infecKous pathogens including bacteria, fungi, and protozoa • Neutrophils are generated at a rate of 1011 per day, which can increase to 1012 per day during bacterial infection. • Researchers once believed that neutrophils were present only during the most acKve phase of an infecKon, funcKoning as pathogen killers.
    [Show full text]
  • How Are White Blood Cells Classified?
    How are white blood cells classified? Copyright 2017 by the Rector and Visitors of the University of Virginia How are white blood cells classified? Types of White Blood Cells: Neutrophil Eosinophil Basophil Lymphocyte Monocyte . The types of white blood cells are shown above. The next page will describe lymphocytes in further detail. A healthy individual has all of these white blood cells types, but within specific ranges. Deviation from these ranges can indicate acute illness or a chronic disease. A mnemonic that is often used to remember the relative amount of each white blood cell that should be present is “Never Let Monkeys Eat Bananas.” Never Neutrophil Highest amounts Let Lymphocyte Monkeys Monocyte Eat Eosinophil Bananas Basophil Lowest amounts . In other words, neutrophils should always be present in higher amounts compared to the other cell types. This will be described further in “A first step in diagnosing LGL leukemia: The blood smear.” Copyright 2017 by the Rector and Visitors of the University of Virginia How are white blood cells classified? Introduction: White blood cells are blood cells that fight infection and disease. Lymphocytes are a type of white blood cell. They can identify antigens (substances foreign to the body) and cause an immune response. There are three types of lymphocytes: T-cell, NK-cell, and B-cell. In healthy adults, 10-15% of the lymphocytes are large granular lymphocytes (LGLs). To learn more about LGL cells, see “A first step in diagnosing LGL leukemia: The blood smear.” A person is diagnosed with LGL leukemia if there is a clonal (copied) population of T-cells or NK-cells present.
    [Show full text]
  • Molecular Profiling of Myeloid Progenitor Cells in Multi-Mutated Advanced Systemic Mastocytosis Identifies KIT D816V As a Distin
    Leukemia (2015) 29, 1115–1122 © 2015 Macmillan Publishers Limited All rights reserved 0887-6924/15 www.nature.com/leu ORIGINAL ARTICLE Molecular profiling of myeloid progenitor cells in multi-mutated advanced systemic mastocytosis identifies KIT D816V as a distinct and late event M Jawhar1,8, J Schwaab1,8, S Schnittger2, K Sotlar3, H-P Horny3, G Metzgeroth1, N Müller1, S Schneider4, N Naumann1, C Walz3, T Haferlach2, P Valent5, W-K Hofmann1, NCP Cross6,7, A Fabarius1 and A Reiter1 To explore the molecular profile and its prognostic implication in systemic mastocytosis (SM), we analyzed the mutation status of granulocyte–macrophage colony-forming progenitor cells (CFU-GM) in patients with KIT D816V+ indolent SM (ISM, n = 4), smoldering SM (SSM, n = 2), aggressive SM (ASM, n = 1), SM with associated clonal hematologic non-mast cell lineage disorder (SM-AHNMD, n = 5) and ASM-AHNMD (n = 7). All patients with (A)SM-AHNMD (n = 12) carried 1–4 (median 3) additional mutations in 11 genes tested, most frequently TET2, SRSF2, ASXL1, CBL and EZH2. In multi-mutated (A)SM-AHNMD, KIT D816V+ single-cell-derived CFU-GM colonies were identified in 8/12 patients (median 60%, range 0–95). Additional mutations were identified in CFU-GM colonies in all patients, and logical hierarchy analysis indicated that mutations in TET2, SRSF2 and ASXL1 preceded KIT D816V. In ISM/SSM, no additional mutations were detected and CFU-GM colonies were exclusively KIT D816V−. These data indicate that (a) (A)SM-AHNMD is a multi-mutated neoplasm, (b) mutations in TET2, SRSF2 or ASXL1 precede KIT D816V in ASM-AHNMD, (c) KIT D816V is thus a phenotype modifier toward SM and (d) KIT D816V or other mutations are rare in CFU-GM colonies of ISM/SSM patients, which might explain at least in part their better prognosis.
    [Show full text]
  • Cellular and Humoral Components of Monocyte and Neutrophil Chen1otaxis in Cord Blood
    Pediat. Res. 11: 677-()1\0 (1977) Chemotaxis neutrophil complement newborn monocytes phagocytes Cellular and Humoral Components of Monocyte and Neutrophil Chen1otaxis in Cord Blood SAYITA G . PAHWA.""'' RAJENDRA PAHWA. ELENA GRII\IES. AND E LIZA13 ETII SI\IITII\VICK Departlllt'lll of Pediatrics aml/nmrwwlogy, Memorial Sloa/1-1\ellcrillg Ca11ca Centa, Nell' York, New York , USA Summary experiment, blood from a healthy adult was tested simultane­ ously. 1\lonoqte and polymorphonuclear neutrophil (J>I\IN) chemo­ taxis was studied in cord blood from healthv term infants. 1\Jono­ ISOLATION OF CELLS c;yte chemotaxis was normal to increased ( 115-126%) whereas PI\IN chemotaxis was decreased (79%) in comparison with that Mononuclear leukocytes were isolated by density gradient of healthy adult l'ontrol subjects. Generation of chemotactic centrifugation on a sodium mctrizoatc-Ficoll solution (Lympho­ factors from cord sera was impaired, being 55% of that gener­ prep. Nyegard and Co., Oslo) (5). The cells were washed three ated by J)(Wied normal human serum (I'NIIS). Cord serum was times and resuspended in RPI\11 (Gibco) supplemented with less inhibitory than pooled adult human serum fur adult mono­ penicillin 50 units, streptomycin 50 Jlg . and glutamine 2 ml\1/ml. qtes when the cells were suspended in HI % serum and tested for As simultaneous analysis of monocytcs by myelopcroxidasc stain chemotaxis. No inhibition of chemotactic factors by either cord and Wright stain were in close agreement. the percentage of or adult sera was observed. The dissociation of chemotactic monocytcs was routinely determined by a myelopcroxidasc stain response of the two diiTerent phagocytic cells may represent a ( 13 ); Wright stain was done to exclude contamination by gra nu­ protecth·e mechanism whereby one cell can compensate for a locytes.
    [Show full text]
  • Systemic Mastocytosis
    Systemic mastocytosis Description Systemic mastocytosis is a blood disorder that can affect many different body systems. Individuals with the condition can develop signs and symptoms at any age, but it usually appears after adolescence. Signs and symptoms of systemic mastocytosis often include extreme tiredness (fatigue), skin redness and warmth (flushing), nausea, abdominal pain, bloating, diarrhea, the backflow of stomach acids into the esophagus (gastroesophageal reflux), nasal congestion, shortness of breath, low blood pressure (hypotension), lightheadedness, and headache. Some affected individuals have attention or memory problems, anxiety, or depression. Many individuals with systemic mastocytosis develop a skin condition called urticaria pigmentosa, which is characterized by raised patches of brownish skin that sting or itch with contact or changes in temperature. Nearly half of individuals with systemic mastocytosis will experience severe allergic reactions (anaphylaxis). There are five subtypes of systemic mastocytosis, which are differentiated by their severity and the signs and symptoms. The mildest forms of systemic mastocytosis are the indolent and smoldering types. Individuals with these types tend to have only the general signs and symptoms of systemic mastocytosis described above. Individuals with smoldering mastocytosis may have more organs affected and more severe features than those with indolent mastocytosis. The indolent type is the most common type of systemic mastocytosis. The severe types include aggressive systemic mastocytosis, systemic mastocytosis with an associated hematologic neoplasm, and mast cell leukemia. These types are associated with a reduced life span, which varies among the types and affected individuals. In addition to the general signs and symptoms of systemic mastocytosis, these types typically involve impaired function of an organ, such as the liver, spleen, or lymph nodes.
    [Show full text]