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FUNNY HAEMATOLOGY Nicolas Novitzky. Leukocytosis  Leukocytosis:  Elevation of leukocyte count above normal for age  Laboratories validate reference.

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Presentation on theme: "FUNNY HAEMATOLOGY Nicolas Novitzky. Leukocytosis  Leukocytosis:  Elevation of leukocyte count above normal for age  Laboratories validate reference."— Presentation transcript:

1 FUNNY HAEMATOLOGY Nicolas Novitzky

2 Leukocytosis  Leukocytosis:  Elevation of leukocyte count above normal for age  Laboratories validate reference ranges in these populations Neonates: 30 x 10 9 /L Adult 10 x10 9 /L  Spurious elevation Normoblasts in severe anaemias Platelet clumps  Examination of blood smear Slide review mandatory in unexplained leukocytosis

3 Lymphoid Leukocytosis Reactive lymphocytosis Not a haematological disorder  Infective mononucleosis syndrome  Range from small to reactive immunoblastas, plasmacytoid and granular lymphocytes  Epstein-Barr virus  Cytomegalovirus infection  HIV primoinfection  Bordetella pertussis  Small immature lymphocytes  Large granular lymphocytes  Toxoplasmosis  Hepatitis B, C  Drug reaction (phenytoin)  Precursors B-cells (haematogones)  Reactive B-cell lymphocytosis

4 Lymphoid Leukocytosis Mononucleosis syndrome  Protracted pyrexia  Weakness  Cervical and generalised adenopathy  Mouth ulcers  CMV colitis, hepatitis  Gillain Barre’ syndrome  Laboratory  Leukocytosis Atypical lymphocytes Absolute lymphocytosis  Positive antibodies (ELISA), PCR, VL EBV 79% CMV 21%  Cold agglutinins  ANA, RhF  Anaemia, thrombocytopenia

5 Malignant Lymphocytosis  Acute lymphoblastic leukaemia  T, B phenotypes  Chronic lymphocytic leukaemia  Lymphoma with spill  Follicular lymphoma 30%  Mantle cell lymphoma  T-cell lymphoma Prolymphocytic leukaemia Sezary syndrome  Lymphadenopathy  Hepato-splenomegaly  Mediastinal adenopathy  Abdominal masses  Laboratory findings  Pancytopenia  Quite typical morphology Monomorphic populations  Monoclonal lymphocytes  Biochemical abnormalities

6 Malignant Lymphocytosis  Abnormal karyotype  Clonal rearrangement of IgH  Single light chain  Chronic lymphocytic leukaemia  Lymphoma with blood spill  Acute lymphoblastic leukaemia

7 Malignant Lymphocytosis  History  Physical exam  Adenopathy common  Splenomegaly in children  Blood cytopenias  Blood tests  Absolute increase in mature cells  Increase in LGLs  Increase in activated lymphocytes

8 Neutrophilic Leukocytosis Neutrophilic leukaemoid reaction  > 35 or 50 x 10 9 /L  Left shift  Infections Chlostridium difficile, Pneumonia, Tuberculosis  Tissue ischaemia  Haemolytic anaemia  Obstetric cases  Epithelial malignancies GM-CSF, G-CSF  Haematological malignancies AML CML MDS / MPS Leukoerythroblastic reaction  Older age, median 75 years  Positive blood cultures  Longer than 1 day LR  No correlation between height of WCC and survival  Hospital mortality 38%  Age > 60 years  Sepsis

9 Neutrophilia  Investigate inflammatory process Treat infection  Investigate malignancy Paraneoplastic Frequently implies disseminated disease Treatment of malignancy  Bone marrow biopsy Specific treatment  Implies bone marrow involvement  Bone marrow biopsy may be diagnostic Granulomata TB, others… Metastatic marrow infiltration Haematological malignancy Leukaemoid reactionLeukoerythroblastic picture

10 Hyperleukocytosis  Haematological malignancy  > 100 x 10 9 /L  Increased mortality May have lineage implications T, B cell, monocytic Increased morbidity Tumour lysis syndrome Disseminated intravascular coagulation Hypercalcaemia, hypo phosphataemia Respiratory failure

11 Hyperleukocytosis  Leukostasis  Mainly affects CNS Confusion Focal deficit Stupor, coma Lungs Hypoxia  Diseases Monocytic leukaemia T, B ALL CML  Mechanism Increased blood viscosity Deformability, volume Endothelial adhesion  Management  Medical emergency  Hydrate properly  Oxygen  Leukapheresis 2 blood volumes (removes 87%) Do not use HES Ca++ supplementation  Repeat if symptomatic  Start chemotherapy Doxorubicin, cytarabine Hydrea

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13 Thrombocytosis  Platelet count > 450 x10 9 /L  Reactive causes most common (90%). Extreme 3-5%  Spurious  Cryoglobulins  Bacteria  Red cell vesicles in burn injury  Reactive  Infection  Chronic inflammation, tissue damage  Malignancy  Haemolysis  Fe deficiency anaemia  Splenectomy  CRP, ESR  IL-1, IL-6, TPO  Ferritin low  High Epo ReactiveLaboratory markers Self limiting Low risk of thrombosis (1.6-4%) Venous Post operative

14 Clonal Thrombocytosis  Chronic myelogenous leukaemia  Ph – t(9;21), BCR/ABL  Essential thrombocythaemia  JAK-2, MPL  Polycythaemia Vera  JAK-2, exon 12, calreticulin  Myelofibrosis  JAK-2, MPL  Microvascular thrombosis Vasomotor symptoms Headaches, syncope, visual Erythromelalgia Elevated thrombexane  Macrovascular disease  Arterial, venous thrombosis Splacnic vessels Mesenteric, portal (25% MPS) Cerebral sinuses  Accumulative risk: 10yr:42% 20yr:52% Risk factors:  Previous thrombosis  Age >65 years Both: 10% per year  Leukocytosis  Erythrocytosis Viscosity Thrombosis major problem

15 Thrombocytosis: Management  Treat primary condition  Fe supplementation  Specific immune therapy  Dispirin  Depending on risk factors  Dispirin  Hydroxyurea  Plateletpheresis  Awareness off bleeding tendency  Acquired vWD ReactiveClonal

16 Myelodysplastic Syndromes Nicolas Novitzky

17 Myelodysplastic Syndromes (MDS)  Clonal hematopoietic Stem Cell Disease  Dysplasia  Ineffective Hematopoiesis (1 or more lines)  Myeloblasts < 20% of all marrow cells  Synonyms :  Dysmyelopoietic syndromes  Preleukemic syndromes  Oligoblastic leukemia  Older adults  median age: 70 years  Primary vs. Secondary MDS (S/P chemotherapy)  Incidence: 3/100,000 n on- age corrected  20/100,000 over age 70

18 MDS: Clinical  Symptoms of Cytopenia  Anemia > Neutropenia +/- Thrombocytopenia  Organomegaly (infrequent)  Hepatomegaly  Splenomegaly Classified according to:  Marrow and blood % blasts  Type and degree of dysplasia  +/- ringed sideroblasts  Cytogenetic abnormalities del (5q)  500 cell diff in marrow  200 cell diff in PB

19 MDS:  Primary  No known history of toxic exposure  Possible etiologies: Virus, Benzene, cigarette (2 fold risk), Fanconi anemia.  Therapy-related  Chemotherapy (alkylating agents)  Radiation Therapy  B12/folate deficiency  Heavy metals (Arsenic)  Congenital dyserythropoietic anemia  Parvovirus B19  GCSF therapy (increased blasts) Differential Diagnosis Etiology

20 Myelodysplastic Syndromes  RA  RARS  RAEB  RAEB-T  CMML  Myelodysplastic Syndromes RA RARS RCMD & RCMD-RS RAEB-1 & RAEB-2 MDS Unclassified MDS del(5q)  Myelodysplastic/Myeloproliferative Diseases CMML Atypical CML Juvenile CMML MDS/MPD, unclassified FAB ClassificationWHO classification

21 MDS: Genetics  5q- syndrome (women, megakaryocyte anomalies  Del 17p (pseudo Pelger-Huet anomaly, therapy related)  Complex cytogenetic (chromosomes 5 & 7) unfavorable prognosis  Del(20q) (erythroid and megakaryocytes)  Abnormal Ch 3 (abnormal megas)

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25 MDS: Prognosis  RA, RC and RARS Normal cytogenetics, del(5q), del(20q) and -Y  RAEB and RCMD  Complex abnormalities  Monosomal karyotype 1, 3, 5, 7, 17  Abnormal Chromosome 7 Low Risk groupHigh Risk

26 Myelodysplastic/Myeloproliferative Diseases (MDS/MPD)  Clonal hematopoietic neoplasms  No history of underlying  CMPD or MDS,  toxins,  Ph’, no del (5q), no t(3;3)(q21;q26), no inv (3)(q21q26)  + finding c/w chronic myeloproliferative diseases,  No Ph’ chromosome  No JAK-2  Spleno & hepatomegaly  Prominent myeloproliferative features  Hypercellular marrow,  Lab and Morphologic features of MDS AND  Dysplasia  Blasts <20%  Mixed MPD and MDS cannot be assigned any other category

27 MDS: Management  Supportive care  Transfusions of blood products  Iron overload  Infections  Specific therapy  Low risk Growth factors  High risk Demethylating agents Stem cell transplantation  Supportive care  Transfusions of blood products  Iron overload  Infections  Specific therapy  Hyroxyurea  azacytidine MDSMPS

28 Conclusion  Medical emergency  Represents serious systemic disorder  Requires urgent investigation  Therapy Antibiotics Cytotoxics Apheresis Treatment of the specific problem  Clonal bone marrow failure  Proper management associated with extended survival Supportive care Demethylating agents HyperleukocytosisMyelodysplasia


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