HEMATOLOGIC DISEASES THE PROCESS OF HEMATOPOIESIS HEMATOLOGIC DISEASES

HEMATOLOGIC DISEASES

THE PROCESS OF HEMATOPOIESIS

HEMATOLOGIC DISEASES RED BLOOD CELL DISORDERS POLY CYTHEMIA WHITE BLOOD CELL DISORDERS ANEMIA QUALITATIVE LEUKOCYTE DISORDERS MULTIPLE MYELOMA LYMPHOMA QUANTITATIVE LEUKOCYTE DISORDERS LEUKEMIA

POLYCYTHEMIA ABSOLUTE ERYTHROCYTOSIS DIABITIC KETOACIDOSIS/ POST SURGICAL DEHYDRATION RELATIVE ERYTHROCYTOSIS LOSS OF TISSUE/ INTRACELLULAR FLUID • POLYCYTHEMIA VERA 3 GROUPS • SEC POLYCYTHEMIA • APPARENT POLYCYTHEMIA

POLYCYTHEMIA VERA • ACQUIRED GENETIC CHANGES IN STEM CELL • RUDDY CYANOSIS CLINICAL FEATURES • HEAD ACHE/ DIZZINESS/ TINITUS • SPLEENOMEGALY • ed R B C & HAEMOGLOBIN DIAGNOSIS • ed BLOOD VISCOSITY & THROMBOSIS • 70% CASES- WBC & PLATELETS

PETICHEAE ECCHYMOSIS VERICOSITIES ON VENTRAL SURFACE GINGIVAL BLEEDING DISCOLOURATION OF MUCOSA ORAL MANIFESTATION DENTAL CONSIDERATION BLEEDING & THROMBOSIS LOCAL HEMOSTASIS BLOOD COUNT Hb BELOW 16 g/dl BEFORE Rx

SECONDARY POLYCYTHEMIA • HI ATTITUDE WITH LOW ATMOSPHERIC PRESSURE • CR PULMONARY DISEASE • RENAL DISEASE • TUMORS • CONG HEART DISEASE HIGH RISK • ENDOCRINE DISORDER • THROMBOSIS /COAGULATION DEFECT

APPARENT POLYCYTHEMIA DISEASE SEEN IN, Hb CONC & PACKED CELL VOLUME NORMAL R B C REDUCTION IN PLASMA VOLUME MIDDLE AGED OBESE MEN HYPERTENSION SMOKING ALCOHOLICS DIURETIC THERAPY

W B C DISORDERS STORED LEUKOCYTE BONE MARROW IN POOLS • MITOTIC POOL NEUTROPHIL • MATURITY POOL • STORAGE POOL 3 ACTIONS MIGRATION PHAGOCYTOSIS BACTERICIDAL

LYMPHOCYTE PRIMARY CELL IN IMMUNITY ‘T’ LYMPHOCYTE ‘B’ LYMPHOCYTE DISEASE OF GRANULOCYTE QUALITATIVE QUANTITATIVE MYELOPROLIFIRATIVE

GRANULOCYTOSIS INCREASED W B C • INFECTION • TISSUE NECROSIS CAUSE • ALLERGIC REACTION • NEOPLASTIC DISEASE • LEUKEMOID REACTION • LEFT SHIFT • MYELOCYTES/ METAMYELOCYTES/ BAND FORMS

GRANULOCYTOPENIA / AGRANULOCYTOSIS GRANULOCYTE NEUTROPHILS NORMAL NEUTROPHIL --- 3000 TO 6000/mm 3 MILD 1000 TO 2000/ mm 3 MODERATE 500 TO 1000/ mm 3 SEVERE BELOW 500 /mm 3 AGRANULOCYTOSIS CAUSE NO NEUTROPHILS IN PERIPHERAL BLOOD DRUG / MEDICATION

CAUSES OF NEUTROPHIL • VIT B 12 AND FOLIC ACID DEFECIENCY • SYSTEMIC LUPUS ERRYTHEMATOSIS • INFECTION • DRUG REACTION “IDIOSYNCRATIC REACTION” • IONIZING RADIATION

LYMPHADINOPATHY MUCOSAL ULCER ACUTE PHARYNGITIS INFECTION LUNG/UT/SKIN CLINICAL FEATURES GEN MALAIZE TREATMENT DISCONTINUE DRUGS CULTURE Br Sp ANTIBIOTICS FEVER CORTICOSTEROID HEMATOPOIETIC STEM CELL TRANSPLANTATION

ORAL / DENTAL CONSIDERATION • ULCERATION OF ORAL MUCOSA • LARGE/ IRREGULAR/ PAINFUL/ ULCER WITH FOUL SMELL • ADVANCED PERIODONTAL DISEASE • PERICORONITIS / PULPAL INFECTION BACTEREMIA • TOPICAL ANTIBIOTIC MOUTH WASH

CYCLIC NEUTROPENIA SEC TO PERIODIC FAILURE OF STEM CELL • TRANSIENT SEVERE NEUTROPENIA - EVERY 21 DAYS • DURING INFANCY / CHILDHOOD • EQUAL IN MALE AND FEMALE • NEUTROPHIL BELOW 500/ mm 3

• FEVER • STOMATITIS • PHARYNGITIS CLINICAL FEATURE • SKIN ABSCESS • INFECTION • LUNG / URINARY TRACT • ULCER • RECTAL / VAGINAl TREATMENT : - INFECTION CONTROL

ORAL / DENTAL CONSIDERATION ORAL ULCER RECUR WITH EACH NEW BOUT OF NEUTROPENIA PERIODONTAL DISEASE RANGE FROM MARGINAL GINGIVITIS TO RAPID BONE LOSS A SERIES OF 3 TOTAL & DIFFERENTIAL W B C COUNT /WEEK FOR 4 TO 6 WEEK MAINTAIN GOOD ORAL HYGIENE

CHEDIAK-HIGASHI SYNDROME • AUTOSOMAL RECESSIVE • OCULOCUTANEOUS ALBINISM • NEUROLOGIC ABNORMALITIES • SEVERE NEUTROPENIA • LARGE BLUE - GRAY GRANULES IN THE CYTOPLASM

• HYPOPIGMENTATION CLINICAL MANIFESTATION • NEUROPATHY & ATAXIS • RECURRENT BACTERIAL INFECTION • LYMPHOHISTEOCYTIC CELL INFILTRATION TREAT THE INFECTION WITH ANTIBIOTICS HEMATOPOIETIC STEM CELL TRANSPLANTATION GINGIVAL & PERIODONTAL DISEASE EARLY LOSS OF TEETH & CARIES

CHRONIC IDEOPATHIC NEUTROPENIA DECREASED NEUTROPHIL PRODUCTION ANTI NEUTROPHIL ANTIBODIES IN THE SERUM CLINICAL FEATURE ASYMTOMATIC RECURRENT BACTERIAL INFECTION NEUTROPHIL BELOW 500/mm 3 ORAL ULCERS SINUSITIS/ PERIRECTAL INFECTION

CORTICOSTEROIDS & CYTOTOXIC DRUGS • PERIODONTAL DISEASE ORAL & DENTAL CONSIDERATION • BONE LOSS / LOSS OF TOOTH / DENUEDED ROOT • GINGIVA IS RED / GRANULOMATOUS MARGIN

LEUKAEMIA VIRCHOW 1874 • MALIGNANCY AFFECTING W B C OF BONE MARROW MALIGNANT CELLS REPLACE/ TURNOFF NORMAL MARROW ANEMIA/ THROMBOCYTOPENIA BODY ORGAN DISTROY NORMAL TISSUE

CLASSIFICATION LEUKAEMIAS ACUTE CHRONIC • LYMPHOBLASTIC • LYMPHATIC • MYELOID

RISK FACTORS • GENETIC DISORDERS DOWNS SYNDROME KLINEFELTERS SYNDROME FACONIS SYNDROME • SIBLINGS • DRUGS & CHEMICALS • SURVIVORS OF ATOMIC BLAST • CHEMOTHERAPHY & RADIATION

ACUTE LEUKAEMIA ACUTE LYMPHOCYTIC ALL ACUTE MYELOGENOUS AML • 65% B LYMPHOCYTES • PRELEUKAEMIC / • 20% T CELL TYPE MYELODYSPLASTIC • 15% NULL CELL LEUKAEMIA SYNDROME

ANEMIA / THROMBOCYTOPENIA INFILTRATION OF LEUKAEMIC CELLS DISSEMINATED INTRAVASCULAR COAGULATION ORAL LESIONS CLINICAL FEATURE CHLOROMAS INFECTIONS OF LUNGS SKIN/ RECTUM/ RESP TRACT URINARY TRACT

LAB DIAGNOSIS: - PERIPHERAL BLOOD BONE MARROW TREATMENT • CHEMOTHERAPY • STAGE - III • SUPPORTIVE CARE • PLATILET TRANSFUSION • HEPARIN • CONTROL INFECTION • IMMUNOSUPPRESSIVE DRUGS

CHRONIC LEUKAEMIA “PRESENCE OF LARGE NO OF WELL DIFFERENTIATED CELLS IN THE BONE MARROW / PERIPHERAL BLOOD & TISSUES & A PROLONGED CLINICAL COURSE EVEN WITHOUT THERAPY” CHRONIC GRANULOCYTIC CGL / CML CHRONIC LYMPHOCYTIC CLL

CHRONIC MYELOCYTIC LEUKAEMIA 90% PT’S HAVE PHILADELPHIA CHROMOSOME CHROMOSOMAL ABNORMALITY AND HEMATOPOIETIC STEM CELL TWO PHASES • CHRONIC PHASE • BLASTIC PHASE DEPLITION OF LEUKOCYTE ALKALINE PHOSPHATASE

SPLEENOMEGALY ELEVATED W B C 30 TO 50 YEARS BONE / ABDOMINAL PAIN CLINICAL FEATURE WEAKNESS FATIGUE DYSNEA ON EXERTION THROMBOCYTOPENIA PETECHIAE ECCHYMOSIS HEMORRHAGE

PHELADELPHIA CHROMOSOMES IN 90% CASES WBC LAB ABSENCE OF LEUKOCYTE ALKALINE PHOSPHATASE BONE MARROW HYPERCELLULAR

TREATMENT • BISULFAN /ALKALATING AGENTS • CHEMOTHERAPHY & RADIATION • MARROW TRANSPLANTATION

CHRONIC LYMPHOCYTIC LEUKEMIA RESULTS IN SLOWLY PROGRESSING MALIGNANCY INVOLVING LYMPHOCYTES • MALES ABOVE 40 YEARS • 90% B LYMPHOCYTES • 5% T LYMPHOCYTES

• SPLEENOMEGALY • HEPATOMEGALY • LYMPHADENOPA THY • ANEMIA INTESTINAL URETHRAL PULMONARY OBSTRUCTION CLINICAL FEATURE INFILTRATE CELLS HYPOGAMAGLOBULINEMIA BACTERIAL/ VIRAL INFECTION

TREATMENT • CHLORAMBUCIL • FLUDARABINE - HI RESPONCE VARIANT OF C L L HAIRY CELL LEUKEMIA 5 : 1 MALE SPLEENOMEGALY / VASCULITIS / ERYTHEMA NODOSUM CLADRIBINE Rx OF CHOICE RARELY INTERFERON & SPLEENECTOMY

DENTAL CONSIDERATIONS LYMPHADENOPATHY INFILTRATE OF LEUKEMIC CELLS OR MARROW FAILURE ORAL BLEEDING HEAD & NECK SIGNS GINGIVAL INFILTRATE ORAL INFECTION ORAL ULCERS

DISEASE & CHEMOTHERAPHY MARROW SUPPRESSION ANEMIA / THROMBOCYTOPENIA ECCHYMOSIS PALLOR GING BLEEDING LOCAL Rx PETECHIAE PLATELET TRANSFUSION RISK HIV HEPATITIS B GRAFT Vs HOST ANTI PLATILET ANTIBODY

ORAL ULCERS DISEASE & CHEMOTHERAPHY ORAL INFECTIONS CANDIDIASIS 7 DAYS AFTER Rx LARGE/ IRREGULAR FOUL SMELL TOPICAL ANTIBACTERIAL PERIODONTAL / PERICORONAL INFECTION WITH OUT NORMAL INFLAMMATION

COMPLICATIONS • DEFECIENT MANDIBULAR DEVELOPMENT IN CHILDRENS • DENTAL AGENESIS • MICRODONTIA • ENAMEL DYSPLACIA • ARESTED ROOT DEVELOPMENT • LICHENOID LESIONS GRAFT-Vs-HOST DISEASE • XEROSTOMIA • KERATOTIC LESIONS • ATROPHY & ULCERATION • DESQUAMATIVE GINGIVITIS

LYMPHOMA B LYMPHOCYTES T LYMPHOCYTES MONOCYTES IMMUNODEFECIENCY RISK FACTOR VIRAL INFECTION CHEMICAL EXPOSURE TWO CATEGORIES • HODGKINS DISEASE • NON HODGKINS LYMPHOMA

HODGKIN’S DISEASE [THOMAS HODGKINS 1832] • VIRAL INFECTION RISK FACTOR • ENVIRONMENTAL EXPOSURE • GENETICALLY DETERMINED HOST RESPONSE CLINICAL STAGING ANN ARBOR STAGING STAGE IV STAGE III

PAIN LESS SWELLING VIRAL & BACTERIAL INFECTION FEVER/ WT LOSS/ PRUIRITIS/ NIGHT SWEATING LYMPHADENOPATHY NON TENDER / RUBBURY CLINICAL FEATURE DELAYED HYPERSENSITIVITY DYSPHAGIA / URETHRAL OBSTRUCTION

LYMPHOCYTE PREDOMINANCE TYPE RYE SYSTEM NODULAR SCLEROSIS TYPE HISTOLOGICAL STAGES MIXED CELLULARITY TYPE LYMPHOCYTE DEPLITION TYPE REED STERNBERG CELLS RADIOTHERAPY / CHEMOTHERAPY DEPENDING ON STAGES

NON HODGKIN’S LYMPHOMA • ARISE FROM ‘B’ OR ‘T’ LYMPHOCYTE • PAINLESS LYMPHADINOPATHY CLINICAL • OLDER THAN 40 YEARS • RENAL OBSTRUCTION/ LIVER OR SKIN INFILTRATION • LESIONS IN WALDEYER’S RING/ G. I. T/ SPLEEN/BONE MARROW • RADIATION / CHEMOTHERAPY - MOST SUCCESSFUL • CYCLOPHOSPHOMIDE+VINCRISTINE+PREDNISONE

DANIS BURKITT 1950 BURKIT’S LYMPHOMA • GEOGRAPHIC ZONE WHERE MALARIA WAS ENDEMIC • CLOSELY LINKED WITH E B V • PRIMARY TUMOR CELL-’B’ LYMPHOCYTE ARISE FROM PEYER’S PATCH/MESENTRIC CLINICAL FEATURE RAPIDLY GROWING TUMOR INCREASE IN SIZE EVERY 1 TO 3 DAYS

TWO CATEGORIES, • SMALL TUMOR BURDEN • LARGE TUMOR BURDEN TREATMENT • CHEMOTHERAPHY [CYCLOPHOSPHAMIDE/ METHOTREXATE/ VINCRISTINE/ CYTARABINE] • REMISSION IN 90% OF CASES

ORAL & DENTAL CONSIDERATION • ASYMTOMATIC CERVICAL LYMPHADENOPATHY • AGENESIS/ HYPOPLASIA/ BLUNTED OR THIN ROOTS • WALDEYER’S RING -COMMON SITE • H. I. V & IMMUNOSUPPRESSIVE DRUGS • MAY PRESENT AS GINGIVAL /TONGUE MASS LESION INTRA OSSEOUS • LOOSE TOOTH/ PARESTHESIA/ SALIVARY GLAND ENLARGEMANT

MULTIPLE MYELOMA ‘MALIGNANT NEOPLASM OF PLASMA CELL -M PROTIEN / BONE LESION/ KIDNEY DISEASE/ HYPERVISCOSITY’ CLINICAL FEATURE M = F/ ABOVE 50 y CLOTTING DEFECT RENAL FAILURE BACTERIAL INFECTION DEPOSITION OF AMYLOID BONE PAIN/ PATHOLOGIC #

Rx: - ALKALATING AGENTS RADIOTHERAPHY [AVERAGE SURVIVAL WITH Rx 2 TO 3 y] • 5 TO 30% JAW LESIONS ORAL MANIFESTATION • SKULL LESION -MULTIPLE RADIOLUCENT LESIONS • IN MANDIBLE ANGLE OF JAW

DENTAL MANAGEMENT • HEMORRHAGE / INFECTION [THROMBOCYTOPENIA/ ABNORMAL PLATELET FUNCTION/ HYPERVISCOSITY/ ABNORMAL COAGULATION] • PLATELET COUNT/ B T/ P T T BEFORE TREATMENT