THROMBOCYTOPENIA

1. THROMBOCYTOPENIA PRESENTED BY: BASIL AL-SAIGH, FMR – 1 SUPERVISORS: DR. ESSALAH DR. RUTHNUM DR. DATTA

2. AGENDA • AN APPROACH TO THROMBOCYTOPENIA (5 STEPS) • 3 CASE REPORTS FROM 4F • PATIENT 1; C/O DR. ESSALAH • PATIENT 2; C/O DR. RUTHNUM • PATIENT 3; C/O DR. DATTA & DR. ESSALAH

3. AN APPROACH TO THROMBOCYTOPENIA (5 STEPS)

4. AN APPROACH TO THROMBOCYTOPENIA • “HOW TO INTERPRET AN ABNORMAL COMPLETE BLOOD COUNT” • MAYO CLINIC PROCEEDINGS JULY 2005; 80(7):923-936 • WWW.MAYOCLINICPROCEEDINGS.COM

5. AN APPROACH TO THROMBOCYTOPENIA CONT’D • KEEP IN MIND THAT USING LOW PLT COUNT TO HELP CLINCH A DX MUST BE IN CONJUNCTION WITH OTHER PEX AND LAB FINDINGS

6. AN APPROACH TO THROMBOCYTOPENIA CONT’D … • STEP 1

7. AN APPROACH TO THROMBOCYTOPENIA CONT’D … • R/O SPURIOUS THROMBOCYTOPENIA (SECOND. TO EDTA-INDUCED PLATLET CLUMPING) • SOLUTION : EXAMINE THE PBS (LOOKING FOR PLATLET CLUMPING) OR REPEAT THE CBC WITH SODIUM CITRATE AS AN ANTICOAGULANT

8. AN APPROACH TO THROMBOCYTOPENIA CONT’D • STEP 2

9. AN APPROACH TO THROMBOCYTOPENIA CONT’D • R/O HUS/TTP/DIC • REASON : THERE IS AN URGENCY FOR SPECIFIC THERAPY IN THESE DISORDERS

10. AN APPROACH TO THROMBOCYTOPENIA CONT’D • WHAT TESTS DO WE ORDER FOR DIAGNOSIS OF HUS/TTP?

11. AN APPROACH TO THROMBOCYTOPENIA CONT’D • CBC & PBS (ANEMIA & SCHISTOCYETES) • SERUM HAPTOGLOBIN (DECREASED) • SERUM LDH (INCREASED) • SERUM CREATININE (INCREASED) • COAGULATION TESTS (EXCLUDE DIC)

12. CASE 1 C/O DR. ESSALAH

13. PATIENT 1 • BACKGROUND • PATIENT 1 • 10 Y/O MALE, OTHERWISE HEALTHY • NON-CONTRIBUTING PMHX, PSHX OR FHX AND NKDA

14. PATIENT 1 • RFC • 09/26/05 - C/O LETHARGY, NON-BLOODY DIARRHEA, LOWER ABD. PAIN, NO APPETITE • 09/28/05 - ABOVE S/S CONT. AND NOW VOMITTING • NAD ON U/S - OPERATD. ON FOR APPEND

15. PATIENT 1 • RFC CONT’D • POST-OP: ANURIC; CATHETERIZED • 09/29/05 : NON-BLOODY DIARRHEA OF SAME FREQUENCY; VOMITTING; DECREASED APPETITE; STILL ANURIC • 09/30/05 : DR. ESALAH CALLED TO ASSESS FOR ANURIA

16. PATIENT 1 • QUESTION • GIVEN THIS CASE PRESENTATION, WHAT IS YOUR DDX FOR PT. 1?

17. PATIENT 1 • DDX • PRE-RENAL FAILURE: SEC. TOVOMITTING AND DIARRHEA • RENAL FAILURE • POST-RENAL FAILURE: BILATERAL URETERAL COMPROMISE IN SURGERY

18. PATIENT 1 • DDX CONT’D • PRE-RENAL FAILURE : PRE-OP VITALS GOOD; PRE-OP IN/OUT GOOD. UNLIKLEY • POST-RENAL FAILURE : OPERATION PERFORMED ON THE RIGHT SIDE OF THE ABDOMOEN SO BILATERAL URETERAL COMPLICATION UNLIKLEY

19. PATIENT 1 • DDX CONT’D • RENAL FAILURE: THE KIDNEY IS COMPOSED OF 4 COMPARTMENTS: • THE BLOOD VESSELS (CONSIDER HUS) • THE GLOMERULUS (CONSIDER GN) • THE TUBULES (CONSIDER ATN) - MCC • THE INTERSTITIUM (CONSIDER DRUGS/OTHER)

20. PATIENT 1 • LABS ON ADMISSION • PLT COUNT : 90 • HGB : 140 • RET COUNT : 144 • LD : 3451 • COAG STUDIES : WNL • UREA : 27.5 • CREAT : 373

21. PATIENT 1 • VIRAL STUDIES • VEROTOXIN : + • SHIG/SALM/C. DIFF/ GP. A STREP : -

22. PATIENT 1 • PATIENT 1 HX RE-VISITED • PRESENTING S/S - MOM NOW STATES THAT PATENT 1 COULD HAVE HAVE SOME EPISODES OF BLOODY DIARRHEA • SOCIAL HX – IN GRADE 6 AND DOING V. WELL IN SCHOOL; MOM TEACHING PRE-SCHOOL @ HOME; NO KIDS INFECTIVE; DAD ENGINEER • DIET – BALANCED DIET; EATS BURGERS OCC. @ FRIENDS HOUSE; LAST ATE STEAK/BURGERS FEW DYS BEFORE ADMISSION AND USED MICROWAVE TOO COOK ITIN

23. PATIENT 1 • WORKING DX OF PATIENT 1: HUS

24. PATIENT 1 • COMPLICATIONS OF HUS • PHUTS • PANCREATITIS • HEMOLYSIS • HEPATIC DYSFUNCTION • HEART FAILURE • UREMIA (RF) • THROMBOCYTOPENIA • SEIZURES/NEUROLOGICAL DEFICITS

25. PATIENT 1 • MANAGEMENT • HUS CAN CAUSE RF • RF CAN CAUSE HYPERKALEMIA, HYPERPHOSPHATEMIA, HYPONATREMIA AND HYPOCALCEMIA : ELECTROLYTE BALANCE AND DIET RESTRICTIONS • RF CAN CAUSE FUID OVERLOAD : FLUID SUPPORT

26. PATIENT 1 • MANAGEMENT CONT’D • RF CAN CAUSE ANEMIA AND LOW PLT. COUNT: BLOOD AND PLT. TRANSFUSIONS • DIALYSIS INDICATED FOR REFRACTORY HYPERKALEMIA OR IF ABOVE FAILS TO CORRECT ELECTROLYTE IMBALANCES, SEVERE ACIDOSIS OR SEVERE UREMIA

27. PATIENT 1 • MANAGEMENT CONT’D • SCREEN FOR LIVER, PANCREATIC DYSFUNCTION • MONITOR FOR PLATELET COUNT, RENAL FUNCTION

28. PATIENT 1 • MGMNT & LABS • DIALYSIS DONE OCTOBER 2ND, 4TH, 6TH, 8TH FOR SIG. ELEVATED UREA AND CREAT LEVELS • UREA : 42.9 - 38 - 17.3 - 25.2 - 22.8 - 18.0 - 12.2 - 6.4 • CREAT : 464 - 623 - 715 - 304 - 266 - 552 - 191 - 73 • PLT COUNT : 74 - 26 - 41 - 101 - 146 - 242 - 449 - 281

29. PATIENT 1 • MGMNT & LABS • HGB : 106 - 82 - 104 - 93 - 107 - 82 - 74 - 76 - 71 • LD : 4098 - 1984 - 1174 • NA AND K : WNL • AMYLASE : 153 - 164 - 113 • LFT : WNL

30. PATIENT 1 • D/C HOME 10/20/05

31. AN APPROACH TO THROMBOCYTOPENIA CONT’D • STEP 3

32. AN APPROACH TO THROMBOCYTOPENIA CONT’D • CONSIDER HYPERSPLENISM • CONSIDER DRUG-INDUCED THROMBOCYTOPENIA

33. AN APPROACH TO THROMBOCYTOPENIA CONT’D • WHAT PEDIATRIC CONDITIONS CAUSE HYPERSPLENISM?

34. BANTI’S

35. AN APPROACH TO THROMBOCYTOPENIA CONT’D • BLOOD FLOW PROBLEM • MOA • INC. SPLENIC VEIN PRESSURE CAUSING CONGESTION • EXAMPLES • SPLENIC VEIN THROMBOSIS EX. TRAUMA, • PORTAL VEIN THROMOSIS FROM HYPERCOAGULABLE STATE EX. PROTEIN C/S DEFICIENCY, NEPHROTIC ETC. • CIRRHOSIS EX. UNTX INB ERROR OF MET, BILIARY ATRESIA, CONGENITAL HEPATITIS • BUDD-CHIARI SYNDROME • CHF EX. UNCORRECTED VALVULAR DEFECTS, PPHN

36. AN APPROACH TO THROMBOCYTOPENIA CONT’D • ANEMIA • MOA • RBC ABNORMALITIES & HYPERPLASIA OF THE RE SYSTEM SECOND TO DESTR OF RBC • EXAMPLES • SCD • HS • THAL

37. AN APPROACH TO THROMBOCYTOPENIA CONT’D • NEOPLASM • MOA • BM HYPOFUNCTION LEADS TO COMPENSATORY EXTRAMEDULLARY HEMATOPOIESIS • EXAMPLES • APLASTIC ANEMIA • MYELOFIBROSIS • LEUKEMIAS

38. CASE 2 C/O DR. RUTHNUM

39. PATIENT 2 • BACKGROUND • PATIENT 2, 3 Y/O FEMALE • TERMS BABY, BORN TO COCAINE-DEPENDANT MOTHER • OTHERWISE HEALTHY • PRODROMAL TONSILLITIS AND ON AMOX X 7 DAYS ON PRESENTATION

40. PATIENT 2 • RFC • 10/16/05 - 1ST NOTED EASY BRUISING FOLLOWING BABY FELL FROM A COUCH • BABY V. IRRITABLE AND HAVING TANTRUMS • MOM DENIES BABY HAS ABD. PAIN • ROS OTHERWISE NON-CONTRIBUTARY

41. PATIENT 2 • RFC CONT’D • GP REFERRED PATIENT 2 TO THE RGH TO R/O HSP

42. PATIENT 2 • PEX • GENERALLY PALE • MULTIPLE BRUISES NOTED ON LIPS, BUTTOCKS, ARMS AND LEGS • MULTIPLE PETECHIAE ON CHEST

43. PATIENT 2 • PEX CONT’D • NOTABLE SPLENOMEGALY 3-4 CM BELOW COSTAL MARGIN • ENLARGED RIGHT PREAURICULAR AND SUBMAXILLARY LN • REST OF EXAM UNREVIELING

44. PATIENT 2 • QUESTION • GIVEN THIS PRESENTATION, WHAT SHOULD YOU CONSIDER IN YOUR DDX?

45. PATIENT 2 • DDX • VASCULITIS EX. HSP • LEUKEMIA • LYMPHOMA • HUS/TTP • CHILD ABUSE

46. PATIENT 2 • LABS • PLT 17 • WBC 75.3 • RBC 2.09 • HGB 65 • MCV 87.5 • LD 1355 • UREA 330 • PT 14.6 • MONO TEST -VE • BLASTS NOTED

47. PATIENT 2 • WORKING DX OF PATIENT 2 : ALL

48. PATIENT 2 • MANAGEMENT • IN ANTICIPATION FOR CHEMO, BABY RECEIVED AN ECHO FOR BASELINE HEART FUNCTION AND URIC ACID LEVELS WERE NOTED TO BE WNL • TRANSFERRED CARE TO PASQUA TO SEE ONCOLOGIST

49. AN APPROACH TO THROMBOCYTOPENIA CONT’D • THYROTOXICOSIS • MOA • T3/4 INDUCED LYMPHOID HYPERPLASIA • EXAMPLES • GRAVES DISEASE

50. AN APPROACH TO THROMBOCYTOPENIA CONT’D • INFECTION • EXAMPLES • MALARIA • MONO • HIV • SARCOID/SLE/SYSTEMIC DZ