Transcript Heme/Onc Review

HEME/ONC
REVIEW
3.8.17
INCREASED
RETICULOCYTE COUNT
& ANEMIA
SS…smear(sickle cells)..Hgb Electrophoresis
HS…Spherocytes..increased osmotic fragility
Coombs + Hemolytic Anemia
Blood Loss…
G6PD deficient patients with oxidant stress
HEMOLYSIS (INCREASED
RETICULOCYTE COUNT)
DIFFERENTIAL
COOMBS + HEMOLYTIC ANEMIA
BP MEDS ALDOMET
HERIDITARY SPHEROCYTOSIS
SICKLE CELL DISEASE
G6PD DEFICIENT RBCS WITH AN OXIDANT STRESS
METAL HEART VALVES
HEMOLYTIC ANEMIA
COOMBS + RETIC 3.7%
LUPUS
BUTTERFLY RASH
HGB 9 INCREASED TO 12
WITH STEROID RX
WORKS AT VECTREN
HX OF LUPUS
RX LOW DOSE STEROIDS
HEREDITARY
SPHEROCYTOSIS WITH
RETIC OF 5% NORMALLY
PARVOVIRUS INFECTION CAN CAUSE PROBLEM WITH RED
CELL PRODUCTION
ACUTE PARVOVIRUS INFECTION CAN PRECIPITATE
WORSENING ANEMIA BECAUSE THE ONGOING
PRODUCTION IS INTERFERED WITH.
ANKYRIN PROBLEM
AUTOSOMAL DOMINANT
SICKLE TRAIT
DISEASE
DECREASED ABILITY FOR URINE CONCENTRATION
INCREASED MORBIDITY WITH EXCESS PHYSICAL ACTIVITY
WITHOUT ADEQUATE HYDRATION
SICKLE CELL DISEASE
THERAPY
HYDRATION IN CRISIS
R/O INFECTION WITH CRISIS
HYDROXYUREA INCREASES HGB F IN CHRONIC STATE
NORMOCHROMIC
NORMOCYTIC ANEMIA
Hgb 10
MCV Normal
Reitic 1.7*
Crest Syndrome, 2 valves
LDH 513
Iron Saturation 11% Low
Anemia secondary to Blood Loss and compensated*
Crest Syndrome
DOG: PEEKAPOO
NORMOCHROMIC
NORMOCYTIC ANEMIA
Hgb 9.1
MCV 96.9 WNL
CREATININE 2.2 INCREASED
LOW ERYTHROPOIETIN
ANEMIA SECONDARY TO CHRONIC RENAL INSUFF*
MICROCYTIC/HYPOCHRO
MIC ANEMIA
DIFFERENTIAL
Iron Deficiency, Chronic Inflammatory Disease, Sideroblastic
Anemia & Thalassemia
Differentiating Tests
• Ferritin(low in Iron Deficiency)
• Fe/Tibc(Low Saturation in Fe Def////>55% in Sidero)
• Marrow (Iron Absent) Iron Deficiency..gold std)
• Hemoglobin Electrophoresis (if Ferritin is normal)
• Hgb A2 increased in Beta Thal Minor(missing one Beta)
• Hgb A2 is normal in Alpha Thal Minor(missing 2 alphas)
MICROCYTIC
HYPOCHROMIA ANEMIA
MCV 60
RBC
• Decreased in Iron Deficiency (eg 3 million)
• Increased in Thalassemia Trait (eg 7 million)
Index
• MCV/RBC
• >13 = Iron Deficiency
• <13= Thalassemia
IRON DEFICIENCY
IRON SATURATION OF <15% WITH LOW TIBC
LOW FERRITIN <15
ABSENT IRON STORES
ABSENT PRUSSIAN BLUE IN MARROW
INCREASED FREE ERYTHROCYTIC PROTOPOPHYRIN
IRON OVERLOAD
HIGH FERRITIN
IRON SATURATION >55%
LIVER IRON IN EXCESS
RX
CHELATING AGENTS FOR TRANSFUSIONS >25
PHLEBOTOMY FOR HEMACHROMATOSIS
B 12 DEFICIENCY
MACROCYTOSIS
CHEMOTHERAPY, FOLIC ACID DEF, B12 DEF, &
WHIPPETS
PANCYTOPENIA CAN OCCUR & IS IMPROVED WITH
REPLACEMENT
PURE VEGANS CAN BECOME B12 DEFICIENT OVER TIME
CLASSIC PERNICIOUS ANEMIA CAN BE TREATED WITH B12
INJECTIONS
SCHILLINGS 1 IS ABNORMAL IN PRENICIOUS ANEMIA
SCHLLINGS 2 IS NORMAL IN PERNICIOUS ANEMIA
ANEMIA
WORKUP..HGB 7.0
NORMAL VITAMINS, CREATININE, NO CHRONIC
INFLAMMATORY DISEASES, LOW RETIC COUNT & NO
CANCER
NEED TO MARROW
MYELODYSPLASTIC
SYNDROME/SIDEROBLAS
TIC ANEMIA
Hgb 9.1
MCV 100
Retic = 1%*
Ferritin 582 increased*
Iron Saturation High*
Marrow:****** Ringed Sideroblasts
RX. EPO
TRANSFUSIONS
CHEMO
ADENOPATHY IN NECK,
CHEST & GROIN
BEGIN CHEMOTHERAPY
TOTAL NODAL RADIATION THERAPY
BROAD SPECTRUM ANTIBIOTICS
TISSUE IS THE ISSUE
HODGKIN’S DISEASE
Reed Sternberg CD30 + Cells
Stages 1-4 A/B
Rad Rx earlier stages
Chemo…Past..MOPP….. NOW ABVD
High Cure Rate
Younger Age group
NON-HODGKIN’S
LYMPHOMA
INTERMEDIATE GRADE
72yo female and Dancer
Left Chest Wall pain and Mass
Biopsy + For NHL Int Grade Diffuse Large Cell Lymphoma
Treatment because of tissue type!!
Therapy
• R-CHOP
NON-HODGKIN’S
LYMHOMA
LOW GRADE/CLL
70yo male with 172 lb White Lab & Vietnam Veteran exposed
to Agent Orange
Sciatica
• WBC Elevated
• Spenomegaly & Adenopathy
Therapy : Symptomatic
NHL LOW GRADE
SYMPTOMATIC WITH
FATIGUE & ANEMIA
Camping and Vietnam Veteran
Fatigue
Hgb 9
B12 deficiency treated and no change in Hgb
All other Vitamin levels normal
Marrow + for Low Grade Lymphoma
Epi- phenomenon M-spike
Therapy
• Symptomatic
NON-HODGKIN’S
LYMPHOMA
88yo male Construction of houses with wife
Intermediate Grade lymphoma 2001 Complete response with
R-CNOP(R-CHOP)
Recurrent Disease Low Grade Lymphoma 2005 to
2017…Watch & Wait
MYELOPROLIFERATIVE
DISEASES
Chronic Myelogenous Leukemia
• 9/22 Genetic Translocation
Essential Thrombocythemia (Jak 2)
Primary Polycythemia Vera(Jak 2)
Myelofibrosis(Jak 2)
CHRONIC
MYELOGENOUS
LEUKEMIA
66 yo retired Pharmacy Tech liked to Camp with Husband
WBC 54,000 with immature cells and not a predominance of
lymphs, platelets & hgb were normal
LAP Score was low
No infection
9/22 Translocation
Interferon 2001
Imatinib(Gleevac) 2002 to 2017
ESSENTIAL
THROMBOCYTHEMIA
83yo USAF
Platelets of 820,000
Iron Normal, No chronic infection or inflammatory Diseases
Jak 2 +
Rx Hydroxurea or Anegralide
Platelets now 518
MCV 118 secondary to Hydroxyurea
PRIMARY
POLYCYTHEMIA VERA
90 yo Baseball Fan, Bucyrus Erie, Conspiracy Theory
Hgb 18.6 in November 2015
• No altitude exposure, epogen use, CT of Abd/pelvis normal
except small cyst in liver
• Erythropoietin level 5.2 and normal, Testosterone level WNL
• JAK 2 +
MYELOMA TRIAD
>20% PLASMA CELLS, LYTIC
LESIONS & M-SPIKE
Most Mature B-Cell in Differentiation
Not Curable
Monitor Efficacy of Therapy with M-Spike Trend
Patient Examples
•
•
•
•
Curtains(77) 2001 to present s/p transplant
UK(85) Triad Stage 3 ..2012 to 2017..nursing home
Nurse (67) 2009 to present Hip Fracture in wheel chair
Wine Glass(61)Sales Rep 2005 to 2017 s/p transplant
MYELOMA
CRAB THERAPY
INDICATIONS
Calcium Elevated from plasma cells activating
osteoclasts/blasts in marrow treated with biphosphanates
Renal Insufficiency from Light Chains
Anemia secondary to Marrow invasion with plasma cells
Bone Lytic Lesions Rx with
Biphosphanates/Chemo/Radiation Rx
ACUTE MYELOCYTIC
LEUKEMIA
>20% BLASTS
MARROW..LOW PLTS/HGB
De-Novo
Predisposing Diseases
• Myelodysplastic (10% blasts) Syndrome (eg Printer)
• Radiation Or Chemotherapy Exposure(Printer)
• Myeloproliferative Diseases(eg Banker/golfer)
ACUTE MYELOCYTIC
LEUKEMIA PATIENT
EXAMPLES
Printer Company Patient & Karate Expert
• Radiation Rx for Seminoma
• Myelodysplastic Syndrome August 2016..10%Blasts
• AML October 2016…>30% Blasts
Golfer/Banker
•
•
•
•
Essential Thrombocythemia 2011-2014
AML >60% Blasts 2014…Complete Remission
Relapse 11/2016 29% Blasts..low platelets/hgb
14% Blast 3/2017
ACUTE LYMPHOCYTIC
LEUKEMIA
Bicyclist
•
•
•
•
•
Fatigue WBC 250k, Hgb 7.8, platelets 19k..82%Blasts
Leukaphoresis prevent CNS problem
Flow confirms diagnosis
Cytogenetics assists with prognosis
Induction/Consolidation not Maintenance because of
Transplant
• CNS THERAPY WITH METHOTREXATE
CHEMOTHERAPY
NEUROTOXICITY
CIS-PLATINUM
TAXOL (PATIENT WITH BREAST CANCER)
ONCOVIN/VINCRISTINE (PATIENT WITH
MEDULLOBLASTOMA)
TAXOTERE
CARDIAC EJECTION
FRACTION TOXITY
MITOXANTRONE
ADRIAMYCIN
HERCEPTIN
HEMATURIA FROM
CHEMOTHERAPY
CYTOXAN
IFOSFAMIDE
THERAPY
MESNA
DECREASED DLCO OR
DIFFUSION CAPCITY
BLEOMYCIN
TUMOR LYSIS
SYNDROME
OCCURS AFTER AGGRESSIVE THERAPY OF
LYMPHOMA/LEUKEMIA WITH HIGH TUMOR BURDEN
INCREASED K, URIC ACID, PHOSPORUS AND CREATININE
DECREASED SERUM CALCIUM
Prevention
Hydration
Allopurinol
Close monitoring for this problem
TUMOR LYSIS SYNDROME
CARCINOID TUMOR
Symptoms
• Flushing, Diarrhea, Palpitations, Abdominal Pain
Elevated
• Serotonin
• 5HIAA
Small Bowel & Lungs are frequent primary sites
Indolent tumor
Rx Embolization & Sandostatin
THYROID CANCER
BEST TO WORST
Follicular
Papillary
Medullary
Anaplastic
(sept 2005)
THERAPY FOR
FOLLICULAR/PAPILLARY
Resection
I 131
Suppression with Synthroid to maintain low TSH
SPINAL CORD
TUMORS
TIME IS CRUCIAL
POSSIBLY
STEROIDS
DECOMPRESSION
STEROIDS
NEUROLOGIC STATUS CRUCIAL AND NEEDS CLOSE
MONITORING & FREQUENTLY IMMEDIATE ACTION
SOME NEUROLGIC COMPROMISES DO NOT REVERSE
BENEFIT OF PET/CT
OVER CT
PET INCREASED ACTIVITY INDICATES
INFECTION
INFLAMATION
TUMOR
PET/CT INDICATES
SIZE OF MASS
CT INDICATES
SIZE BUT NOT ACTIVITY OF MASS
TREATABLE
IMMUNODEFIENCY
LOW IGM
LOW IGA
LOW IGG**
NUCLEAR CATASTROPHE
IN CHERNOYBL
DECREASED LEVELS OF LYMPHOCYTEs
INDICATE PROBLEMS WITH SURVIVAL AND WILL
HELP IN TRIAGE
PHLEBOTOMY
THERAPY
REMOVE BAD HUMORS
HEMACHROMATOSIS
MAKE NEARLY IRON DEFICIENT
POLYCYTHEMIA VERA
MAINTAIN HGB 15/HCT 45
PORPHYRIA CUTANEA TARDA
IMPROVES SKIN COMPLAINTS
GLIOBLATOMA
MULTIFORME
RESECTION/RADIATION/CHEMO IS STANDARD OF
THERAPY
SIMILAR FOR ASTROCYTOMA
PROGNOSIS
OLIGODENDRIGLIOMA> ASTROCYTOMA> GLIOBLASTOMA
MULTIFORME
BRAIN CANCER
MOST COMMON FROM METASTASIS
BREAST
LUNG
RENAL
PRIMARY BRAIN CANCER LESS FREQUENT THAN FROM
METASTASIS
CERVICAL CANCER
SQUAMOUS CELL CANCER IS MOST COMMON
ADENOCARCINOMA IS RARE
HPV TESTING INDICATED
PAP SMEAR IS INDCATED TO PREVENT THIS PROBLEM OR
DIAGNOSE IT AT AN EARLY STAGE
LOCALIZED THERAPY CAN BE CURATIVE
CONIZATION IS AN OPTION FOR EARLY STAGE DISEASE
WITH THE HOPES OF FUTURE PREGNACNY
ENDOMETRIAL
CANCER
ADENOCARCINOMA IS MOST COMMON
PERI/POST MENOPAUSAL WOMEN
TAMOXIFEN IS A RISK FACTOR
POST MENOPAUSAL BLEEDING SHOULD BE PROMPTLY
EVALUATED
HIGH CURE RATE
GASTRIC CANCER
INCREASED INCIDENCE IN JAPAN
INCIDENCE IS DECREASING IN USA
H. PYLORI IS A RISK FACTOR
BLADDER CANCERS
85% ARE SUPERFICIAL
TOBACCO INCREASES THE RISK
TRANSITIONAL/UROTHELIAL IS MOST COMMON IN USA
SQUAMOUS CELL CANCER IS MORE COMMON IN AREAS
INFECTED WITH SCHISTOSOMA HAEMATOBIUM
DEEP LESIONS REQUIRE RADICAL CYSTECTOMY
RADIATION THERAPY
TO NECK
INDICATED
HEAD & NECK CANCER
SOME THYROID MALIGNANCIES
LYMPHOMA…HODGKIN’S/NON-HODGKIN’S
LONG TERM SIDE EFFECTS
XEROSTOMIA
DENTAL PROBLEMS
HYPOTHYROIDISM
SARCOMA
BONES OR SOFT TISSUE
RESECTION WITH NEGATIVE MARGINS
RADIATION THERAPY TO PREVENT LOCAR RECURRENC
LUNG METS CAN BE RESECTED
BLEEDING
PROBLEMS..CARDINAL
PATIENT
ECCHYMOSIS MULTIPLE
TIMES
Elevated PTT (35,55,37, 36 for patient)
• Intrinsic Pathway
•
•
•
•
•
XII
XI
IX
VIIIc Ecchymosis… Factor 8 was 13% Average
VIIIVWF
• Symptoms
• Mucosal…Platelet Problem VIIIvwf
• Ecchymosis/Joint Bleed VIIIc/IX
PTT 1:1 DILUTION
CORRECTS INDICATES SOME DEFICIENCY IN THE
INTRINSIC PATHWAY
FACTORS XII, XI, IX, VIIIC & VIII VWF ARE
POSSIBITIES
1:1 DOES NOT CORRECT
INHIBITOR
DIC/LUPUS ANTICOAGULANT
PT/INR
EXTRINSIC PATHWAY
FACTOR VII
EFFECTED BY VITAMIN K
ABSENCE OF VII WILL INCREASE THE PT
COUMADIN WILL INCREASE THE PT/INR
½ LIFE OF VII IS THE SHORTEST AND WHY THE INR IS
TESTED ON PATIENTS
B CELL DIFFERNTIATION
OF MALIGNANT CELLS
EARLIEST IN DIFFERENTIATION HAVE THE MOST IN S
PHASE & ARE THE MOST CURABLE
WILM’S TUMOR
PEDIATRICIAN EXAMS ABDOMEN
ABDOMINAL MASS IS PALPABLE
VERY TREATABLE