DrVivian Barrera / Dr FarhanJune 20, 2013
 91 y.o. Chinese female Premorbidly ADL assisted Ambulant with WF Independent in grooming and feeding BARTHEL 12/20...
 DM HBAIC : 6.1 13/5/13 HYPERTENSION HYPERLIPIDEMIA TB OF KIDNEYS 1980 Bleeding GIT 2009 -extensive diverticulardis...
 6/5 /2013 Spiked fever (+) cough Bibasal creps (+) SOB , SP02 89%-95% CXR: no consolidation/ effusion CPR 1 TW 6.8 P...
 Oriented TPP Non toxic looking Neck : supple H : S1 S2 C : bibasal creps A : soft , no palpable bladder C: supple ...
 Hb 9.3 MCV 98.2 H MCH 32.4 H MCHC 33 TW 5.9 Plt 258 Crea 179 H Urea: 6.7 TCalcium 2.09L Cor Ca 2.35 iPhosphate...
 TSH 12.9 T4 0.719 Hbaic 6.1 Albumin 25 CK Normal LFT: Normal APTT /PT Normal UFEME 23/48/0 (-) protein (+) est...
 Fracture Distal shaft of fifth Metatarsal bone L Xray toes: diffuse bony osteopenia, healing fracture of distalshaft o...
 23/5 Urine PCR 2.07 UFEME 20/270/0 (+) protein (+) esterase ANA screen negative ENA screen negative DsDNA <25 ANCA...
 Patient doesn’t qualify for DDX of MM Crea -->Prob post TB Kidneys and CRPD M band is just 3G/L Calcium is normal An...
 There is no evidence of lytic lesions in skullor the bones to suggest presence of MMdeposits Unlikely MM, (?) likely MG...
Patient Multiple Myeloma Tuberculosiskidney/chestMGUSAge 91 older adultMA >60* mean age >70Anemia * * Not seenDeranged Cre...
 Diagnosed only if Serum Monoclonal Protein( IgA, IGg, IgM) < 3g/dl) Clonal BM plasma cells < 10% Absent of lytic lesi...
 Past TB of kidney Discordant kidney size Recurrent or resistant urinary tract infection, sterile pyuriawith or without...
 Older age Anemia , leukopenia, low albumin Unexplained renal dysfunction Multiple fractures Elevated b2 macroglobuli...
 1% of all cancers 10% of all hematologic cancer F>M (1.4.:1) Incurable Disease of older adults ( mean age: 66y.o) S...
 Complex , poorly understood Establishment of a limited clonalproliferation Progression of MGUS to MM
 Genetic predisposition Older age Immunosupression Hormonal Environmental exposures Radiation Herbicides, insectici...
 Anemia 73 % Bone pain 58% Inc creatinine 48% Fatique 32% Hypercalcemia 28% Weight loss 24 % Paresthesias 5% Hepat...
 Presence of M –protein in serum No specific level of m-protein is used as a cut offvalue 40% percent of patients with ...
1. Hypercalcemia: serum calcium>2.75 mmol/L2. Renal dysfunction: serum creatinine>173 mmol/L3. Anemia: hemoglobin 2 g/dL b...
 FBC, blood chemistry Electrophoresis immunofixation Quantitative Immunoglobulin Xrays, Ct scan, MRI, PET BMA B2 Mi...
 Deferred treatment if asymptomatic Bisphosphonates Blood transfusion or erythropoeitin Chemotherapy Autologous stem ...
 Stage I B2 M < 3.5g/dl, albumin >/ 3.5g/dl Stage II B2M < 3.5mg/dl, alb <3.5g/dl or B2M 3.5—5.5 mg/L irrespective of...
Stage 1 Stage 1 Stage 2 Stage 3HB > 10g/dl < 8.5mg/dlcalcium Normal > 12 mg/dlSkeletal survey: NormalSingle plasmacytoma o...
 Myeloma screen : M Band 3/G/L IgG 5.88 N IgA: 9.31 Increased IgM 0.21 N K/L ratio: O.o6 decreased Kappa free light...
 Urine electrophoresis Protein electrophoresis Monoclonal band detected and bands in alpha 1,alpha 2, beta and gamma gl...
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  • Patient was admitted in ht=this isntitutio last march -- fro fracture, was dischrage to AMKCH for rehab. On 6the pf may, patient dtated to
  • The heart size cannot be assessed on this projection...... Calcified old granulomas... CXR: calcified old granulomas on both upper lung fields and left mid lung zone
  • Since patient h was difficulty weaning from 02, rpt xray done 5 Days later. The hear size is enlarge, prominent hilum is likely due to vascular shadows, fribrocalcific foci in bilateral upper znes and left mid zone are in keeping with previous granulomatous infection, ther is blunting of costophrenic angle, suggestive of bilateral pluerl effusin. Referral to pulmologistr was done.. Bedside ultrasound was done which showed no tapable pleural effsuin
  • In view of elevated creatinine, it was trended. It was initially thought as AKI, hence hydration was done, then... But it reached up to 300 (+0 mg /dl Renal consult was done.
  • Renal AKI w/ leucocytomia and past hx of TB kidney T?RO vasculitis Ddx: chronic interstitial nephritis or acute interstitial nephritis Small left kidney may be sec to tb 2. Pnemnia 5 th metatarsal fracture Myeloperoxidase deficiency  is a hereditary deficiency of the enzyme, which predisposes to  immune deficiency . [7] Antibodies  against MPO have been implicated in various types of vasculitis, most prominently crescentic glomerulonephritis and Churg-Strauss syndrome. They are detected as perinuclear ANCAs (p-ANCAs), as opposed to the cytoplasmic ANCAs (c-ANCAs) against proteinase-3 (PR3), which are strongly associated with Wegener&apos;s granulomatosis. P; IV HYDRATION DAILY RENAL PANEL MYELOMA TESAT TB AFB ABD CULTURE
  • Unexplained renal insuffiency suggests possible invlvement by the light chains due to MGUS, MM or related malignacy Diagnosis of DGUS is olny considered of the following 3 criteria is considered... SO ANY PATIENT WITH A NON Igm MONOCLONAL protein&gt; 3 or with &gt;10 % CLONAL PLASMA CELLS IN BONE MARROW DOES NOT HAVE mgus. However, could this patient ..........
  • ANOTHER DDS IS ---TB of the kidneys. Genitourinary tuberculosis (GUTB) is the second most common form of extrapulmonary tuberculosis after lymph node involvement . Kidney is usually the primary organ infected in urinary disease, and other parts of the urinary tract become involved by direct extension. The GUTB has varied presentation and some of the common ways are: Recurrent or resistant urinary tract infection, sterile pyuria with or without hematuria . Irritative voiding symptoms, i.e., frequency, urgency, and dysuria.An incidental diagnosis in a known case of tuberculosis. Renal (hydronephrosis/pyonephrosis) or epididymal mass. Infertility and pelvic inflammatory disease. Renal failure (Chronic kidney disease due to parenchymal infection and obstructive uropathy. The various other ways of presentation described are: flank pain with acute pyelonephritis, non-healing wounds, sinuses, or fistulae (nephrocutaneous fistula or vesicovaginal fistula), and hemospermia. The most common symptoms with which the patients have presented are in the form of irritative voiding, which are found in more than 50% of the patients. The other symptoms in GUTB can be fever, weight loss, anorexia, backache, and abdominal pain.[
  •   unexplained renal dysfunction, anemia, or pathologic fracture should prompt evaluation for this diagnosis. . a clearly elevated B2 macroglobulin in the absence of renal faliure or an inflammatory process would suggest a diagnosis of MM and the patient should be carefully examined.
  • pATHOBIOOLOgy of MM is a complex prc3ess of malignant clone of plasma cell origin. Virtually all MM myeloma cases are preceded by a premalignant plasma cell prolifereation disorder of the Monoglonal gammopathy of undetermined significance. MGUS is present in over 3 percent of thhe population above 50 and prgresses to Myeloma or arelated malignancy at a rate of 1 % per year
  • Epidemiologic data suggest a genetic predisposition as well as other potential risk factors including… Radiologists exposed to large doses of long term radiation have an increased risk of MM Workes in nuclear plants and farmers who use herbicieds, insecticides and those who employ benzene and petroleum products have increased risk of MM but the evidence is not compelling.
  • A restospective analysis of 1027 sequential patients diagnoised with MM at a single instituion found the following symproms and signs at presentaiton.
  • Symptomatic hyperviscosity……..
  • Bisphosphonates are given to prevent fracture , routine administered to prevent fractures, they also have been observed to have direct anti tumor effect in patients without known skeletal disease. Chemotherapy Autologous SCT: - not curative but prolong overall survival and complete remission Allogenic SCT- has potential cure but is ony avialable to asmall percentage
  • This staging is only in patients who meet diagnostic criteria of myeloma. Patients with MGUS and aymptomatic myeloma who have renal dysfuntion from unrelated causes such as diabetes or hypertension may have elevated b2M levels just from the renal dysfunction and cannot be considered as stage III myeloma.This is one of the drawbacks of the Iss. It daoes not really quantify quantity tumor burden or extent unlike staging used in other cancers. It is recommended to use along durie salmon staging system.
  • Staging and classification of multiple myeloma A staging system is used by the medical profession to describe how advanced the myeloma is, and how it is affecting the body. A common staging system is called the Durie-Salmon system. The four factors of the Durie-Salmon system: What is the red blood cell count? How much calcium is in the blood? How much paraproteins (monoclonal proteins, or M proteins) is in the blood? The general state of the patient&apos;s bones. Stage one (Durie-Salmon system) Blood calcium levels are normal Red blood cell levels are either normal or slightly below normal Low levels of paraproteins (monoclonal proteins, or M proteins) in the blood Bones are either undamaged or slightly damaged Most patients have no symptoms Stage two (Durie-Salmon system) Red blood cell levels still relatively normal Calcium blood levels still relatively normal Levels of paraproteins (monoclonal proteins, or M proteins) still low Damage is present in one or two bones Stage three (Durie-Salmon system) Red blood cell levels have fallen. Symptoms of anemia are probably present. Blood calcium levels have risen. Symptoms of hypercalcemia are likely. High levels of paraproteins (monoclonal proteins, or M proteins) in the blood. Damage to three or more bones. The Durie-Salmon system also has a system to indicate the health or deterioration of the kidneys: A - kidneys are either undamaged or only slightly damaged. B - kidneys are damaged, kidney function is abnormal. Kidney damage is more likely during stages two or three, but can also occur in stage one.
  • PLAN REFER TO HEMATOLOGY SINGLE KIDNEY: CONTRAINDICATION FOR RENAL BIPSY
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    1. 1. DrVivian Barrera / Dr FarhanJune 20, 2013
    2. 2.  91 y.o. Chinese female Premorbidly ADL assisted Ambulant with WF Independent in grooming and feeding BARTHEL 12/20 AMT 6/10 Social Widowed 1 adopted daughter :Main carer
    3. 3.  DM HBAIC : 6.1 13/5/13 HYPERTENSION HYPERLIPIDEMIA TB OF KIDNEYS 1980 Bleeding GIT 2009 -extensive diverticulardisease - Antral erosions Fracture Right IT 4 S/PDHS 2006 Fracture left IT s/P PFNAin 2010 Fall 5/2013 with LeftOlecranon fracture S/P ORIF left Olecranon Left Humerus Fracture▪ Conservative management▪ Discharged to AMKCH for rehab▪ BMD: T score -2.2 2010
    4. 4.  6/5 /2013 Spiked fever (+) cough Bibasal creps (+) SOB , SP02 89%-95% CXR: no consolidation/ effusion CPR 1 TW 6.8 PLT: 203 crea 98 UFEME: suggestive of UTI Urine CS: Proteus Given IV Rocephine, Flagyl 10/5/2013 Repeat bloodsHB 8.8 TW 5.9 crea 187, Na 140 K 3.7
    5. 5.  Oriented TPP Non toxic looking Neck : supple H : S1 S2 C : bibasal creps A : soft , no palpable bladder C: supple bilaterally DRE: Brown stools, No blood Motor 5/5 uLE
    6. 6.  Hb 9.3 MCV 98.2 H MCH 32.4 H MCHC 33 TW 5.9 Plt 258 Crea 179 H Urea: 6.7 TCalcium 2.09L Cor Ca 2.35 iPhosphate 1.36 Vit B12 209 Folate : 5.20 Transferrin 1.29 Ferritin 270.7 Iron serum 12.1 Iron sat 42% Haptoglobin 157 Vit d 5ug/L CRP 8.0 Procalcitonin 0.27 PTH 19.68
    7. 7.  TSH 12.9 T4 0.719 Hbaic 6.1 Albumin 25 CK Normal LFT: Normal APTT /PT Normal UFEME 23/48/0 (-) protein (+) esterase Urine CS: NBG Sputum CS: normalflora AFB Smear: negativex3 takings
    8. 8.  Fracture Distal shaft of fifth Metatarsal bone L Xray toes: diffuse bony osteopenia, healing fracture of distalshaft of the fifth metatarsal bone noted Seen By ORTHO Casting done
    9. 9.  23/5 Urine PCR 2.07 UFEME 20/270/0 (+) protein (+) esterase ANA screen negative ENA screen negative DsDNA <25 ANCA Anti MPO negative Anti PR3 negative
    10. 10.  Patient doesn’t qualify for DDX of MM Crea -->Prob post TB Kidneys and CRPD M band is just 3G/L Calcium is normal Anemia not completely worked up▪ for skeletal survey▪ B2 Macroglobulin
    11. 11.  There is no evidence of lytic lesions in skullor the bones to suggest presence of MMdeposits Unlikely MM, (?) likely MGUS Needs Further anemia WU Not for BMA BETA 2 MACROGLOBULIN 14,392
    12. 12. Patient Multiple Myeloma Tuberculosiskidney/chestMGUSAge 91 older adultMA >60* mean age >70Anemia * * Not seenDeranged Creatinine * * Not seenChronic renalparenchymal disease* Not seenCalcium normal * *Low VIT DOsteopenia*Absent skeletal lyticlesions* * *Infection * *AFB NegativeB2 microglobulin Poor prognosis
    13. 13.  Diagnosed only if Serum Monoclonal Protein( IgA, IGg, IgM) < 3g/dl) Clonal BM plasma cells < 10% Absent of lytic lesions. Anemia, hypercalcemia,renal insufficiency ( end organ damage) that canbe attributed to plasma cell proliferative disorder
    14. 14.  Past TB of kidney Discordant kidney size Recurrent or resistant urinary tract infection, sterile pyuriawith or without hematuria . Renal (hydronephrosis/pyonephrosis) Renal failure (Chronic kidney disease due to parenchymalinfection and obstructive uropathy Infertility and pelvic inflammatory disease. Urine AFB smear ( 55% sensitivity) Urine AFB culture ( 41% sensitivity)Indian J Urol. 2008 Jul-Sep; 24(3): 401–405.
    15. 15.  Older age Anemia , leukopenia, low albumin Unexplained renal dysfunction Multiple fractures Elevated b2 macroglobulin M proteins Raised IgA, ( 20% of MM) Protein electrophoresis Raised K/L freeLight chains ( 20% of MM) Immunofixation : (+) monoclonal bands
    16. 16.  1% of all cancers 10% of all hematologic cancer F>M (1.4.:1) Incurable Disease of older adults ( mean age: 66y.o) Small but unknown fraction of cases arefamilial Evolve from MGUS
    17. 17.  Complex , poorly understood Establishment of a limited clonalproliferation Progression of MGUS to MM
    18. 18.  Genetic predisposition Older age Immunosupression Hormonal Environmental exposures Radiation Herbicides, insecticides, benzene, petroleum
    19. 19.  Anemia 73 % Bone pain 58% Inc creatinine 48% Fatique 32% Hypercalcemia 28% Weight loss 24 % Paresthesias 5% Hepatomegaly 4 % Slenomegaly 1% Lympadenopathy 1 % Fever 0.7%
    20. 20.  Presence of M –protein in serum No specific level of m-protein is used as a cut offvalue 40% percent of patients with symptomatic MMwill have an M protein of < 3 g/dl Presence of 10% clonal bone marrow plasmacells Related organ or tissue impairment CRAB
    21. 21. 1. Hypercalcemia: serum calcium>2.75 mmol/L2. Renal dysfunction: serum creatinine>173 mmol/L3. Anemia: hemoglobin 2 g/dL below lower limit ofnormal4. Lytic bone lesions (CT and MRI may be used toidentify suspicious findings on plain films)5. Symptomatic hyperviscosity6. Amyloidosis7. Recurrent bacterial infections (more than twoepisodes in 1 year)
    22. 22.  FBC, blood chemistry Electrophoresis immunofixation Quantitative Immunoglobulin Xrays, Ct scan, MRI, PET BMA B2 Microglobulin
    23. 23.  Deferred treatment if asymptomatic Bisphosphonates Blood transfusion or erythropoeitin Chemotherapy Autologous stem cell transplant Allogenic SCT
    24. 24.  Stage I B2 M < 3.5g/dl, albumin >/ 3.5g/dl Stage II B2M < 3.5mg/dl, alb <3.5g/dl or B2M 3.5—5.5 mg/L irrespective of serum albumin Stage III B2M >/ 5.5 mg/dl
    25. 25. Stage 1 Stage 1 Stage 2 Stage 3HB > 10g/dl < 8.5mg/dlcalcium Normal > 12 mg/dlSkeletal survey: NormalSingle plasmacytoma orosteoporosis3 or more lseionsSerum paraproteinlevel< 5 g/dl If IgG<3 g/dl id IgA 7 g/dl 5 g/dl 9.31Urinary lightchains excretion< 4 g/24h > 12 g/24Survival rate 62 mos 45 mos 29 mos
    26. 26.  Myeloma screen : M Band 3/G/L IgG 5.88 N IgA: 9.31 Increased IgM 0.21 N K/L ratio: O.o6 decreased Kappa free light chain: 37.9 increased Lambda free light chain : 607 increased
    27. 27.  Urine electrophoresis Protein electrophoresis Monoclonal band detected and bands in alpha 1,alpha 2, beta and gamma globulin regions seen Immunofixation electrophoresis Monoclonal band detected with anti lambda Total protein urine 1.94g/L
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