We present two patients with initial presentation of myeloma plasmocyticum and symptoms of chronic myeloid disease in peripheral blood and bone marrow cytology. [journals.viamedica.pl]

Clinical history 31 year old female with a history of an acute myeloid leukemia (M5b FAB), diagnosed one year prior to the presented bone marrow biopsy. [iupui.edu]

Presentation on theme: "Chronic Myeloproliferative Neoplasies (CMPN)"— Presentation transcript: 1 Chronic Myeloproliferative Neoplasies (CMPN) 2 Myeloid Neoplasies WHO Classification Myeloproliferative neoplasies (MPN) Myelodysplastic syndromes (MDS) [slideplayer.com]

CML – Chronic phase Peripheral Smear • Anaemia-NCNC • WBCis elevated (2,00,000/cummm ) • Blast if present 18. [slideshare.net]

[…] feature in 25% § Arterial (2/3) § Increased if p re-existing vascular disease / risk factors § V enous (1/3) § M esenteric, splenic, hepatoportal thr o mboses are not uncommon presentations § M icrovascular (eg. [ihaematology.com]

• Splenectomy

  Management of Polycythemia Vera andEssential Thrombocythemia,ASH Education Book 2005,p 31 Causes of thrombocytosis ET and other MPD Iron deficiency anemia Splenectomy or hyposplenism Malignancy Collagen vascular disease Infection Hemolysis or bleeding [slideplayer.com]

  Patients with painful, massively enlarged spleens refractory to myelosuppressive therapy are occasionally treated with radiation therapy, but they may ultimately require splenectomy. [emedicine.medscape.com]

  Clinical signs: age: usually affects middle-aged and older adults (median is approx. 55 years of age) splenomegaly bicytopenia or pancytopenia prognosis: good, log-term remissions following chemotherapy or splenectomy Histology: Intersticial infitration [atlases.muni.cz]

  […] haemorrhage and myelofibrotic transformation, but less venous thrombosis § Interferon alpha - a s for PV (possibly first line if age § Anegralide § Need to check ECG / Echo prior to commencing treatment § Busulphan, radioactive phosphorus, alkylating agents § Splenectomy [ihaematology.com]

• Pathologist

  Edited by four leading surgical pathologists - Noel Weidner, MD, Richard J. Cote, MD, Saul Suster, MD and Lawrence M. [books.google.com]

  CONCLUSIONS: Because CIMF has a variety of clinical presentations, diagnosis may be challenging; the prefibrotic stage of CIMF has always been a challenging disease for pathologists to diagnose accurately. [thedoctorsdoctor.com]

• Inflammation

  JAK2V617F (50%) or cMPL mutation (MPLW515L and MPLW515K) + or no evidence of reactive thrombocytosis 36 37 Diagnosis of ET: WHO 2008 (2) Exclude other causes of thrombocytosis Reactive Anemias Iron deficiency Hemolytic Acute blood loss Post splenectomy Inflammation [slideplayer.com]

  RT was associated with inflammation (3), bleeding or iron deficiency anemia (2) and herbal medication (1). After the average follow-up of 27 months (range 0~133 months), the median OS was not reached ( Figure 1A ). [kjim.org]

• Swelling

  Clinical signs: frequent infections hepatosplenomegaly lymphadenopathy or tonsil swelling in approximately 50% of cases leukocytosis in peripheral blood usually 20 – 30×10 9 /l with monocytosis, BCR/ABL negative, less than 20% blasts in peripheral blood [atlases.muni.cz]

• Hyperactivity

  Inherited predispositions and hyperactive Ras in myeloid leukemogenesis. Pediatr Blood Cancer 2006; 46 : 579–585. 110. Pardanani A, Hood J, Lasho T, Levine RL, Martin MB, Noronha G et al. [nature.com]

Polycythemia Vera Workup. Medscape Drugs and Diseases. Available online at http://emedicine.medscape.com/article/205114-workup#c5. Accessed on 1/22/17. Lal, A. (2016 November 10 Updated). Essential Thrombocytosis Workup. Medscape Drugs and Diseases. [labtestsonline.it]

See Workup for more detail. [emedicine.medscape.com]

[…] examination signs relate to the CMPD itself and include hepatomegaly, splenomegaly, conjunctival injection, facial plethora, skin excoriations, and gouty arthritis and tophi. 34 Imaging The following chest imaging modalities are typically performed during a workup [phaonlineuniv.org]

• Thrombocytosis

  The group includes chronic myelogenic leukaemia, polycythaemia vera, essential thrombocytosis and primary myelofibrosis. [tidsskriftet.no]

  The seven conditions were chronic granulocytic leukaemia (now chronic myeloid leukaemia), polychthaemia vera, agnigeic myeloid metaplasia (now primary myelofibrosis), thrombocytosis (essential thrombocytosis), megakaryocytic leukaemia and erythroleukaemia [allaboutblood.com]

  Management of Polycythemia Vera andEssential Thrombocythemia,ASH Education Book 2005,p 31 Causes of thrombocytosis ET and other MPD Iron deficiency anemia Splenectomy or hyposplenism Malignancy Collagen vascular disease Infection Hemolysis or bleeding [slideplayer.com]

  This group includes chronic myelomonocytic leukemia, juvenile myelomonocytic leukemia, atypical chronic myeloid leukemia, refractory anemia with ringed sideroblasts and thrombocytosis (RARS-T) and unclassified MDS/MPD. [ingentaconnect.com]

  These disorders include polycythemia rubra vera, essential thrombocytosis, chronic myeloid leukemia, and idiopathic myelofibrosis. Myeloproliferative disorder A disorder in which the bone marrow produces too many cells too rapidly. [medical-dictionary.thefreedictionary.com]

• Philadelphia Chromosome Positive

  chromosome positive chronic myelogenous leukemia developing in a patient with acute lymphoblastic leukemia Ponatinib in Patients (pts) with Chronic Myeloid Leukemia (CML) and Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia (Ph+ ALL) Resistant [documents.mx]

  Chronic MPD Polycythemia vera, agnogenic myeloid metaplasia, primary thrombocythemia, chronic myelogenous leukemia–Philadelphia chromosome positive or negative, chronic monocytic leukemia, chronic neutrophilic leukemia myeloproliferative disorder Any [medical-dictionary.thefreedictionary.com]

  ''Myeloproliferative disorders(MPD) are relatively rare chronic hematologic malignancies that include the classic myeloproliferative disorders: Philadelphia chromosome-negative Philadelphia chromosome-positive Essential Thrombocythemia (ET ) Chronic Myelogenous [cmleukemia.com]

  Agents used in the treatment of the disease include the following: Imatinib mesylate (Gleevec): Approved for use in Philadelphia chromosome–positive chronic myelogenous leukemia patients in chronic phase; also indicated for chronic myelogenous leukemia [emedicine.medscape.com]

  (MF) Polycythemia Vera (PV) Philadelphia chromosome-positive Chronic Myelogenous Leukemia (CML) There are atypical Myeloproliferative Neoplasm Disorders which include molecularly defined PDGFRA-rearranged eosinophilic/mast cell disorders; PDGFRB-rearranged [mpninfo.org]

• Neutrophilia

  ’s § A1: HCT >0.6 men, >0.56 women or raised red cell mass § A2: Absence of Jak 2 mutation § A3: No cause of secondary erythrocytosis § A4: palpable splenomegaly § A5: acquired genetic abnormality (excluding Bcr-Abl) § B1: thrombocytosis > 450 § B2: neutrophilia [ihaematology.com]

  There may be cytopenias affecting the other cell lines: there may be neutropenia, thrombocytopenia, neutrophilia, monocytosis, thrombocytosis. [patient.info]

  […] blast crisis (acute leukemia): myeloblasts exceed 20% (AML), sometimes transformation into acute lymphoblastic leukemia (ALL) or biphenotypic/bilinear leukemia 3.5.2.5 Chronic Neutrophilic Leukemia (CNL) Introduction: A rare disorder characterised by neutrophilia [atlases.muni.cz]

• Erythroblast

  erythroblasts + young myeloid cells* *(promyelocytes, myelocytes, meta, band and polys.) 47 48 IMF: LAB LDH, uric acid: increased Bone marrow : Aspiration : Dry tap Biopsy: Increased fibrosis and megakaryopoesis JAK2 mutation:50%** ** Kenneth Kaushansky [slideplayer.com]

  A2 + B1 + 1 other B or A1 + A2 + 4B’s (if not B1) § A1 = diffuse marrow fibrosis § A2 = No Philadelphia chromosome § B1 = splenomegaly § B2 = anisopoikilocytosis with tear drop poikilocytes § B3 = circulating immature myeloid cells § B4 = circulating erythroblasts [ihaematology.com]

The median survival for polycythemia vera is more than 10 years with treatment. [ajmc.com]

Treatment for this disease is palliative. [emedicine.medscape.com]

Resolution of chronic hypoxemia in pediatric sickle cell patients after treatment with hydroxyurea Splenic irradiation in the treatment of patients with chronic myelogenous leukemia or myelofibrosis with myeloid metaplasia : Results of daily and intermittent [documents.mx]

Data on combined treatment in patients who have MPNs with recurrent thrombosis are lacking. [hematologyandoncology.net]

Indications EXJADE is indicated for the treatment of chronic iron overload due to frequent blood transfusions (≥7 mL/kg/month of packed red blood cells) in patients with beta thalassemia major aged 6 years and older. [exjade.com]

Clinical signs: pathological bone fractures anemia hypercalcemia monoclonal gammopathy (AL amyloidosis) prognosis: bad, an incurable disease, median survival time is 3 years Complex diagnostic criteria — Durie-Salmon staging system. [atlases.muni.cz]

Patients with Chronic Neutrophilic Leukaemia usual have mild splenomegaly with good prognosis 25. [slideshare.net]

The blast crisis transformed from unclas-sified MPD had a grave prognosis as it responded poorlyto chemotherapy. [documents.tips]

Spivak reports receiving consulting fees from Incyte and holding a patent on a genetic assay to determine prognosis in patients with polycythemia vera (PCT/US2013/069192). [nejm.org]

Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]

Etiology: Most often unclear. [atlases.muni.cz]

15 PRV: Etiology-Pathophysiology Progenitors in PRV gain clonal predominance and supress the proliferation of normal progenitor cells. [slideplayer.com]

Epidemiology: 0.4 – 1.3 / year Age: Median (wide age distribution) Survival: median:3-5 years 43 (Idiopathic Myelofibrosis) (Myelofibrosis with Myeloid Metaplasia) bone 44 IMF Fibroblasts are not part of the clonal process Megakaryocyte derived cytokines [slideplayer.com]

Currently, these diseases are not measured by the Surveillance, Epidemiology, and End Results Program of the National Cancer Institute. [ajmc.com]

EPIDEMIOLOGY The incidence of CMPD is rare and estimated between 6 and 9 new cases per 100,000 per year, most commonly occurring between 40 and 60 years of age. 6, 7 There have been several publications showing that prevalence of PH in CMPD ranges from [phaonlineuniv.org]

Epidemiology ACML is a rare disorder of old adults. No predominance of sex. The incidence is not established. Clinics Anemic syndrome. Splenomegaly. Malaise. Cytology Peripheral blood: Leukocytosis with a high count of immature granulocytes. [atlasgeneticsoncology.org]

Epidemiology of myelodysplastic syndromes and chronic myeloproliferative disorders in the United States, 2001–2004, using data from the NAACCR and SEER programs. Blood. 2008;112(1):45–52. [ehoonline.biomedcentral.com]

15 PRV: Etiology-Pathophysiology Progenitors in PRV gain clonal predominance and supress the proliferation of normal progenitor cells. [slideplayer.com]

"Myeloproliferative neoplasms: molecular pathophysiology, essential clinical understanding, and treatment strategies". J. Clin. Oncol. 29 (5): 573–82. doi : 10.1200/JCO.2010.29.8711. PMID 21220604. 2 ^ a b Vardiman, J. W.; Thiele, J.; Arber, D. [en.wikipedia.org]

Increased epo § Idiopathic erythrocytosis § Exclusion criteria met but not positive criteria Polycythaemia vera § 2-3/ 100 000 § Median age of onset is 55-60 years § M:F: 1:1 § Expansion of total red cell mass +/- increased platelets, neutrophils § Pathophysiology [ihaematology.com]

Pathophysiology of Blood Disorders. New York: McGraw-Hill; 2011. http://accessmedicine.mhmedical.com.ezproxy.library.ubc.ca/content.aspx?bookid=676&Sectionid=44827790. Accessed February 09, 2014 [bloodcancerclips.wordpress.com]

Genetic basis and molecular pathophysiology of classical myeloproliferative neoplasms. Blood. 2017;129:667-679. Rumi E, Cazzola M. Diagnosis, risk stratification, and response evaluation in classical myeloproliferative neoplasms. [labtestsonline.it]

Treatment of Myerloproliferative Neoplasm The goals of therapy of myeloproliferative neoplasia are Prevention and treatment of complications of increased cell counts Prevention of progression of MPN Erradicating the malignant clone Prevention and treatment [allaboutblood.com]

Get optimal test results with guidance on error detection, correction, and prevention as well as cost-effective test selection. [books.google.com]

[…] postpolycythemic myelofibrosis and extramedullary hematopoesis Life expectancy: > 10 years Less than normal age/sex group Thrombosis Transformation AML MF 28 PRV Treatment Venesection to keep Hct: < %45 Low dose aspirin (if it’s not contraindicated) Prevent [slideplayer.com]