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SPLEEN Professor Anwar Sheikha THE SPLEEN MAN IN THE SPLEEN LAND PHAGOCYTOSIS OF PARTICUALTE MATTERS IMMUNOLOGICAL FUNCTIONS OF THE SPLEEN REGULATION OF BLOOD POOLING ERYTHROPOIESIS EMH Main Police & Security Force ﻣﺮﻛﺰﺷﺮﻄﺔ ﻮﺃﻤﻥﺍﻠﺑﻠﺪ SEWERAGE DISPOSAL ﺍﻠﻣﺠﺎﺮﻱ FUNCTIONS OF THE SPLEEN GRAVEYARD اﻠﻤقبرة ﺍﻷحتياط EMH POOLING اﻠﻤخزﻥ ﻣﺮﻛﺰﺷﺮﻄﺔ ﻮﺃﻤﻥﺍﻠﺑﻠﺪ ﺍﻠﻣﺠﺎﺮﻱ FUNCTIONS OF THE SPLEEN اﻠﻤخزﻥ ﺍﻷحتياط اﻠﻤقبرة ﭙﻮﻠﻳﺲﻮﺋﻪﻤﻧﻰ ﻨاﻮﺷﺎﺮ FUNCTIONS زبلﻮ ﺋﺎﻮﻩﺮﻮ OF THE SPLEEN ساﻳﻠﻮ ﺍحتياط ﮔﻮﺮستان SPLEEN MYSTERII ORGANUM PLENUM EXTRACTION OF MELANCHOLIC HUMOURS GREAT LAUGHTERS HAVE GRAET SPLEENS HEMATOLOGIST SURGEON ? WHO SPLEEN GALEN’S ORGAN OF MYSTERY IMMUNOLOGIST INFECTIOUS DISEASE PHYSICIAN WHO SHOULD SEE A PATIENT WITH SPLENOMEGALY? WILLAIM CRSOBY ROMANCING THE SPLEEN HAS BEEN A PLEASURE, BUT MARRIAGE WAS OUT OF THE QUESTION SPLENOMEGALY KALA AZAR HYDATID PARASITIC MALARIA ACUTE TRYPANOSOMA INFECTIONS S.B.E. TYPHOID PARATYPHOID TYPHUS INEFCTIOUS MONONUCLEOSIS INFECTIOUS HEPATITS BRUCELLA TOXOPLASMA SEPTICEMIA SUBCUTE CHRONIC T.B. MILD SMG BRUCELLA SYPHILIS HISTOPLASMA CHRONIC MENINGEAL SEPTICEMIA LATE IN REGRESSIVE SPLENOMEGALY EARLY IN PROGRESSIVE SPLENOMEGALY MILD SMG ITP DISORDERS WITH SARCOID OCCASIONAL SPLENOMEGALY SLE MYELOMA FELTY AMYLOID CHRONIC IRON DEFICIENCY ANEMIA MEGALOBLASTIC ANEMIA LYMPHOMAS ACUTE LEUKEMIA HEMOLYTIC ANEMIA PRV MODEARTE SMG PORTAL HTN WITH CONGESTIVE SMG CLL MARKED SMG MYELOFIBROSIS THAL. CML CYSTS TUMORS GAUCHER TSS PARASITIC BILHARIZIA KALA AZAR CAUSES OF SPLENOMEGALY HYPERPLASIA EMH LYMPHOMA CONGESTIVE PATHOGENESIS OF SPLENOMEGALY RED CELL POOLING INFLAMMATORY STORAGE MISCELANEOUS NEOPLASIA Leukemias (AL; CML; CLL) Lymphomas Metastasis EMH HA H. S. H.E. Thal INFECTIONS Bacterial Parasitic V.L. TSS Bilh. Thalassemia Osteopetrosis Myelofibrosis SMG STORAGE DISEASES Gaucher; NPD Histiocytosis Mucopolysac. CONGESTIVE Cirrhosis TSS TROPICAL SPLENOMEGALY SYNDROME MALARIAL SPLENOMEGALY TSS MALARIAL SPLENOMEGALY TROPICAL SPLENOMEGALY SYNDROME MAJOR DIAGNSOTC CRITERIA TSS MINOR DIAGNSOTC CRITERIA Gross Splenomegaly Hepatic Sinusoidal lymphocytosis Immunity to Malaria Normal Immune response to Antigenic Challenge High Serum IgM Normal PHA Response Clinical & immunological Response to Antimalarial Hypersplenism Lymphocytic Proliferation Occurrence in families CLINICAL FEATURES OF TSS SYMPTOMS Abdominal Swelling Abdominal Pain Cough Weakness Leg Swelling Epistaxis Hernia % SIGNS 65 Massive splenomegaly Hepatomegaly 50 13 12 10 5 5 Pallor Jaundice Hemic Murmur Leg Ulcer Hernia % 100 90 30 20 5 5 2 PATHOGENESIS OF TSS MALARIA PARASITE B- LYMPHOCYTE x IgM ++++ HIGH M. Wt. IMMUNE COMPLEXES SPLENOMEGALY DACIE’S SYNDROME IDIOPATHIC NON-TROPICAL SPLENOMEGALY DACIE’S SYNDROME * A 45 YEAR OLD FARMER * 3 MONTHS H/O TIREDNESS & NIGHT SWEATS * O/E PALLOR & GROSS SPLENOMEGALY RADIONUCLIDE STUDIES: RCM: 13.8 ml/Kg Splenic Red Cell Pool: 28% T50 RBC Survival: 22 days Plasma Volume: 60 ml/Kg Hb: 53 g/L WBC: 1,900/Ul Plat. 52,000/ul Marrow: Active SPLENECTOMY Weight: 1570 gm NO SPECIFIC FEATURES 17 YEARS LATER PATIENT WAS NORMAL WITH NORMAL CBC I Ashkenazi Jews ADULT “NON-NEUROPATHIC” SPLENOMEGALY HYPERSPLENISM PORTAL HTN II INFANTILE GAUCHER’S DISEASE ↓ β– GLUCOCEREBROCIDASE III JEUVENILE “NEUROPATHIC” EARLY DEATH PREDOMINANTLY NEUROPATHIC RHEUMATOID ARTHRITIS SPLENOMEGALY 10% FELTY’S SYNDROME ANEMIA TP LEG ULCERS INFECTIONS NEUTROPENIA 1% PIGMENTATION PUL. HTN GALL STONES SERIOUS BACTERIAL INFECTION IS RELATIVELY UNCOMMON SPLENECTOMY DOES NOT ALTER COURSE OF FELTY’S HYPERSPLENISM SPLENOMEGALY CYTOPENIA ANEMIA NEUTROPENIA THROMBOCYTOPENIA HYPERSPLENISM GRAVEYARD CORRECTION O F THE CYTOPENIA AFTER SPLENECTOMY HYPERCELLUALR OR NORMOCELLULAR BONE MARROW ABBATOIRE SPLENOMEGALY CYTOPENIA ANEMIA NEUTROPENIA THROMBOCYTOPENIA ﮔﻮﺮﺳﺗﺎﻦ HYPERSPLENISM CORRECTION O F THE CYTOPENIA AFTER SPLENECTOMY HYPERCELLUALR OR NORMOCELLULAR BONE MARROW ﮔﺮﺩﻯ ﺷﻪﻫﻳﺩﺍﻦ SPLENOMEGALY CYTOPENIA ANEMIA NEUTROPENIA THROMBOCYTOPENIA ﻣﻗﺑﺮﺓ HYPERSPLENISM CORRECTION O F THE CYTOPENIA AFTER SPLENECTOMY HYPERCELLUALR OR NORMOCELLULAR BONE MARROW ﻤﺟﺰﺮﺓ HEMATOLOGICAL GI CELIAC D. HERPETIFORMIS ULCERATIVE COLITIS CIRRHOSIS T. SPRUE SICKLE E.T. AUTOIMMUNE HYPOSPLENISM CTD SLE RA CAH MISCELANEOUS SURGICAL “SPLENECTOMY” IRRADIATON AMYLOIDOSIS SARCOIDOSIS GvHD CONGENITAL INDICATIONS FOR SPLENECTOMY USUALLY SOMETIMES OCCASIONALLY H.S. AIHA MF CHRONIC ITP ACUTE ITP CLL HYPERSPLENISM LYMPHOMAS HCL H.E. THAL. MAJOR FELTY’S SURGICAL INDICATIONS DIAGNSOTIC LYMPHOMA STAGING FOR SPLENECTOMY ITP HS THERAPEUTIC HCL HE CDA MF H.A. CML HYPERPLENISM Thal. & HbSS AIHA Pitted Red Cells OPSI Overwhelming Post-Splenectomy Infection OPSI ORGANISMS ENCAPSULATED BACTERIA Strep. Pneumonaie H. influenza tyoe b N. meningitides OPSI RATE TRAUMA 1.5% HEMATOLOGICAL 3.5% PORTAL HTN 8.2% HODGKIN 10% SICKLE 15% THALASSEMIA 25% OPSI RATE AGE (YEARS) OPSI 1 - 16 9-20% <1 >1 <5 >5 <1 >1 H.S. 50% 2.8% 8.1% 3.3% 21% 3.5% Prevention of OPSI Avoid splenectomy when possible Delay splenectomy until patient is >5 yr Immunize before splenectomy with pneumococcal vaccine, meningococcal vaccine & Hib vaccine Daily prophylactic antibiotic administration (penicillin or amoxicillin orally or erythromycin for allergic patients) for all high risk patients of development of sepsis (i.e., younger patients, underlying disease) Aggressive education of patient and family to ensure rapid medical attention & Antibiotics Partial rather than total splenectomy Obtain MedicAlert or equivalent warning system Antibiotic Doses for Prophylaxis for Patients with Asplenia/Hyposplenia Age Pencillin V Amoxicillin Erythromycin (EES)a 2 mo to 3 yr 125 mg p.o. b.i.d. 125 mg p.o. b.i.d. 125 mg p.o. q.i.d. 3 yr and older 250 mg p.o. b.i.d. 250 mg p.o. b.i.d. 250 mg p.o. q.i.d. Emergency Management of Febrile Asplenic Patients Temperature below 38.5ºC Examine patient (vital signs, localizing findings, especially any indication of meningitis). Obtain blood culture and other cultures as indicated. Administer stat intravenous or intramuscular antibiotics (ceftriaxone, ampicillin, or equivalent coverage). Begin oral antibiotics to cover encapsulated organisms (choice depends on local epidemiology and whether the patient is receiving prophylactic antibiotics). Temperature above 38.5ºC Examine patient (vital signs, localizing findings, especially any indication of meningitis). Obtain blood cultures, CBC & other cultures as indicated. Administer stat i.v. antibiotics (ceftriaxone, ampicillin, or equivalent coverage). Maintain intravenous hydration and observe patient for at least 6-12 hr in medical facility. Consider hospital admission, depending on local circumstances, ability to monitor patient, and proximity to medical facility. Discharge requires a responsible adult immediately available to bring patient back in event of change in clinical stat A CASE STUDY SHAYKHA MOH’D ASIRI AGE: SEX: NATIONALITY: HOSP. #: 70 YEARS FEMALE SAUDI 014450 MARKED SPLENOMEGALY MILD PANCYTOPENIA ? MYELOFIBROSIS HISTORY: Admitted Muharam 1409 to KCH 3/12 H/O NO Generalized Weakness Inability to take full meals Marked Abdominal Distension Fever Sweating Wt. Loss Pruritis Bone Pain Bleeding Cough Sputum Chest Pain No Lymphadenomegaly PAST HISTORY SOCIAL HISTORY FAMILY HISTORY NIL OF NOTE PRESUMPTIVE DIAGNOSIS IN THE REFERRING HOSPITAL MYELOFIBROSIS WEIGHT O/E Small, old, Frail & Cachectic Lady Mild Pallor No Jaundice 44 Kg Abdomen: Huge Spleen occupying almost all of the Abdomen Chest, CVS, CNS, etc: Unremarkable MCV: Retic. Smear: Coomb’s: Clotting: Malaria: INVESTIGATIONS Hb: WBC: Platelets: Neutrophils: 101 5,400 90,000 2,000 g/L /uL /uL /uL BLOOD BIOCHEMISTRY: TOXOPLASMA BRUCELLA BILHARIZIA LEISHMANIA SEROLOGY 84 Fl 6% OK Negative Normal Negative NORMAL NEGATIVE SERUM PROTEIN ELECTROPHORESIS: IMMUNOGLOBULIN QUANTITATION: ANF: NORMAL NORMAL NEGATIVE BONE MARROW: NOT DIFFICLUT ASPIARATION: ACTIVE; LYMPHOID CELLS <15% BIOPSY: ACIVE & HYPERCELLULAR NO EVIDENCE OF MYELOFIBROSIS LYMPHOID CELLS: NOT INCREASED RADIOLOGY Chest X-Ray: Abdominal Ultrasound: Normal Huge Spleen No Focal masses Kidneys, Liver & GB: Normal CT SCAN: NO MEDIASTINAL WIDENING MARKED SPLENOMEGALY NO ABDOMINAL LYMPHADENOMEGALY DIAGNOSIS ? ? MYELOFIBROSIS SMEAR NORMAL RETICULIN MARROW EASY NORMAL SUMMARY: AN OLD CACHECTIC LADY MASSIVE SPLENOMEGALY MILD HYPERSPLENISM DIAGNOSTIC & THERAPEUTIC SPLENECTOMY SPLEEN: GROSLLY ENLRAGED WEIGHT: 2400 GRAMS FINELY NODULAR MICROSCOPY: NON-HODGKIN’S LYMPHOMA FOLLICULAR SMALL CELL NODULAR PRIMARY LYMPHOMA OF THE SPLEEN POST-OPERATIVE MANAGEMENT ? ? CHEMOTHERAPY WAIT & WATCH FOUR YEARS LATER SHE PUT ON 10 KILOGRAMS NO EVIDENCE OF LYMPHOMA CURED TEN YEARS LATER SHOULD I HAVE USED CHEMOTHERAPY? THANK YOU