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Hyperviscosity Syndrome 
By 
Mohamed AbdElmotaal Safa 
Ass. Lecturer of Internal Medicine 
Faculty of Medicine 
Tanta University
PPhhyyssiioollooggiiccaall bbaacckkggrroouunndd
Viscosity is a property of 
liquid that exhibits its resistance 
to the flow of one layer over 
. another 
Blood as a circulating tissue 
is composed of fluid plasma 
, and cells (red blood cells 
(.white blood cells, platelets 
For blood to be viscid affection of its component should 
.Occure
PLASMA 
(to 90% 85( 
CELLS 
(to 15% 10( 
RBCs .Plat WBCs 
Polycyathemia 
Thrombocytosis 
Leukemia 
Monoclonal 
Polyclonal 
HHVV ooff BBlloooodd 
MMMM 
WWMM 
MMGGUUSS 
11rryy AAmmyyllooiiddoossiiss 
HHeeaavvyy cchhaaiinn 
ddiisseeaassee 
IInnffeeccttiioonnss 
TTuummoorrss 
LLiivveerr ffaaiilluurree 
CCoollllaaggeenn ddiisseeaassee 
SSaarrccooiiddoossiiss
PPaatthhoopphhyyssiioollooggyy 
HV of blood leads to 
vascular stasis and 
resultant hypoperfusion 
then lead to the clinical 
symptoms HVS.
Epidemiology 
AGE 
SEX 
MORTALITY 
More in old age 
More in males 
Related to 
cause &complications
Epidemiology 
Data are non conclusive 
due to lake of 
informations 
AGE 
SEX 
MORTALITY 
More in old age 
More in males 
Related to 
cause &complications
CCLLIINNIICCAALL PPIICCTTUURREE 33××33 
Mucosal bleeding 
Visual changes 
Neurological symptoms 
Cardiopulmonary 
symptoms 
Renal symptoms 
&Dermatological 
. Constitutional sym
Mucosal bleeding 
• SSppoonnttaanneeoouuss gguumm bblleeeeddiinngg 
• EEppiissttaaxxiiss 
• RReeccttaall bblleeeeddiinngg 
• MMeennoorrrrhhaaggiiaa 
• PPeerrssiisstteenntt bblleeeeddiinngg aafftteerr mmiinnoorr 
procedures
VViissuuaall cchhaannggeess 
rraannggee ffrroomm bblluurrrreedd vviissiioonn ttoo vviissiioonn lloossss.. 
Change in visual acuity: (BlurringDiplopiaVisual 
loss) 
Characteristic “link-sausage effect” on funduscopy 
Alternating bulges and constrictions within the 
retinal 
veins 
Retinal hemorrhage, detachment 
Exudate, microaneurysm formation 
Papilledema
NNeeuurroollooggiiccaall ssyymmppttoommss 
FFrreeqquueenntt&&VVaarriiaabbllee 
• VVeerrttiiggoo 
• HHeeaarriinngg lloossss 
• PPaarreesstthheessiiaass 
• AAttaaxxiiaa 
• HHeeaaddaacchheess 
• SSeeiizzuurreess 
• SSoommnnoolleennccee pprrooggrreessssiinngg ttoo ssttuuppoorr aanndd 
ccoommaa
CCaarrddiiooppuullmmoonnaarryy 
ssyymmppttoommss 
CCoonnssttiittuuttiioonnaall 
ssyymmppttoommss 
Dyspne 
Dysrhythmias 
Hypoxia 
Heart failure 
Fatigue 
Lethergy 
Anorexia
DDeerrmmaattoollooggiiccaall 
RReennaall ssyymmppttoommss 
RRaayynnaauudd pphheennoommeennoonnoo 
LLiivveeddoo rreettiiccuullaarriiss 
PPaallppaabbllee ppuurrppuurraa 
EErruuppttiivvee ssppiiddeerr nneevvuuss––lliikkee 
DDiiggiittaall iinnffaarrccttss 
PPeerriipphheerraall ggaannggrreennee 
Nephritic or 
nephrotic syndrome 
hematuria 
Sterile pyuria
PPeeaarrll ttoo bbee 
hhiigghhlliigghhtteedd 
To early diagnose a patient 
with HVS you should have 
high index of suspicion for 
it 
in any patient with 
unexplained 
MMuuccoossaall bblleeeeddiinngg 
((NNoossee&&gguumm)) 
NNeeuurroollooggiiccaall CCOO 
…………HHeeaaddaacchhee 
BBlluurrrriinngg ff vviissiioonn 
DDyyssppnneeaa 
EEssppeecciiaallllyy tthhoossee wwiitthh uunnddeerrllyyiinngg bblloooodd ddiisseeaassee
PPhhyyssiiccaall EExxaammeennaattiioonn 
Related to the cause : spleanomegaly &flushed 
face in PRV 
:Related to organ affected 
Bruises, epistaxis, or gum bleeding may be noted 
Ophthalmic examination :decreased visual acuity,dilated retinal 
veins, "sausage-linked"of the retinal veins, or retinal Hge 
, Neurologic examination may reveal various abnormalities 
including diminished mental status, confusion, ataxia, or 
nystagmus 
Cardiopulmonary examination may reveal signs of CHF with 
(volume overload (rales, LLO,CNV, and hypoxia
DIAGNOSTIC WORKUP
HHiissttoorryy && CClliinniiccaall 
eexxaammeennaattiioonn 
Lab. Investigations 
Imaging
HHiissttoorryy && CClliinniiccaall 
eexxaammeennaattiioonn 
Put in mind any Pt presented with 
especially if unexplained and the 3×3 
Pt is suspected to have underlying 
hematological disease
. Put in mind lab • 
Comment on the • 
P t samples if it • 
is 
difficult in • 
manipulations • 
and 
separation •
Lab. clues 
CBC with blood film 
Globulin gap 
Measurement of serum 
viscosity 
Metabolic panel and 
Electrolyte 
Urine analysis 
Coagulation profile 
Important markers 
SPEP and SPIF
CBC with blood film 
Erythrocytosis 
Leukemia 
Thrombocytosis 
N N anemia with 
rouleaux formation
Globulin gap 
Consider measuring total protein 
,TP) and albumin) 
; as in the paraproteinemias 
a globulin gap 
TP – albumin = 4 or greater) may ) 
exist
Measurement of serum viscosity 
Ostwald viscosimeter 
Normal range for the 
serum viscosity relative 
.to water is 1.4–1.8 
Minimal viscosity at 
which symptoms 
develop is 4.0 centipoise 
(.cp )
:Metabolic panel and Electrolyte 
Renal dysfunction is commonly 
noted in HVS 
Hypercalcemia and pseudohyponatremia 
in MM
: Urine analysis 
Proteinuria 
Hematuria 
Sterile pyuria 
BJP 
Protein Electrophoresis
Coagulation profile 
For any coagulopathy 
workup 
…PT,PTT,BT)
Important markers 
LDH, B2 Microglobulins, serum 
vit B 12 
Uric acid and ALP.
SPEP and SPIF
Tailor additional workup • 
according 
• to patient presentation
Imaging 
Bone survey 
Us abdomen and Pelvis 
CT &MRI brain 
CXR 
ECHO cardiography
TREATEMENT
Pre-hospital Stabilization Therapy 
A,B,C Care with keeping 
IV fluid axis(Rehydrate with 
0.9% NS IV fluid.) 
Avoid blood transfusion ?
Emergency Departement Treatment 
plasmapheresis 
Hydration 
Early apheresis 
and phlebotomy 
Standard therapies 
For CHF, bleeding… 
platletpheresis 
leukapheresis 
Erythrocytosis
Be Wise enough☺ 
Be careful in p RBCs transfusion even 
when needed (v.slow) 
Diuretic may worsen the condition 
TLS can occure easily with leukapheresis 
IDA can occure from repeated venesiction 
and lead to microcytosis with more HV 
Phlebotomy with hydration (in non 
anemic Pt)may be clue Till Apheresis is 
available
Note that the definitive 
treatment of HVS is 
treatment of the 
underlying disorder 
eg, chemotherapy). If) 
the underlying disease 
process is left untreated 
. the HV will recur,
Prognosis 
:It depends on 
Severity of the complications, 
The underlying cause 
Response of the appropriate definitive 
treatment.
NNooww…….. MMeennttaall bbrreeaakk !! 
......إإننسسوواا 
......إإننسسوواا 
......إإننسسوواا
HVS is a clinical entity that can be fatal but can also 
be 
easily treated if early diagnosed 
For health care supervisors to diagnose HVS they 
must 
Have high index of suspicion towards Unexplained 
mucosal bleeding dyspnea and coma 
Blood film is very important and may be clue for 
many 
case 
Keep in mind lab. Comments on your patient 
samples 
.If it is repeatedly condense and block lab. Machines 
Be wise enough in judging patients with HVS even 
in 
Treating current problem you may worsen the matter
SSuurree yyoouu aarree 
ttiirreedd...... 
EEnnoouugghh 
......!!PPlleeeeeeeeeezz

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Hyperviscosity syndrome

  • 1. Hyperviscosity Syndrome By Mohamed AbdElmotaal Safa Ass. Lecturer of Internal Medicine Faculty of Medicine Tanta University
  • 2.
  • 3.
  • 5. Viscosity is a property of liquid that exhibits its resistance to the flow of one layer over . another Blood as a circulating tissue is composed of fluid plasma , and cells (red blood cells (.white blood cells, platelets For blood to be viscid affection of its component should .Occure
  • 6. PLASMA (to 90% 85( CELLS (to 15% 10( RBCs .Plat WBCs Polycyathemia Thrombocytosis Leukemia Monoclonal Polyclonal HHVV ooff BBlloooodd MMMM WWMM MMGGUUSS 11rryy AAmmyyllooiiddoossiiss HHeeaavvyy cchhaaiinn ddiisseeaassee IInnffeeccttiioonnss TTuummoorrss LLiivveerr ffaaiilluurree CCoollllaaggeenn ddiisseeaassee SSaarrccooiiddoossiiss
  • 7. PPaatthhoopphhyyssiioollooggyy HV of blood leads to vascular stasis and resultant hypoperfusion then lead to the clinical symptoms HVS.
  • 8. Epidemiology AGE SEX MORTALITY More in old age More in males Related to cause &complications
  • 9. Epidemiology Data are non conclusive due to lake of informations AGE SEX MORTALITY More in old age More in males Related to cause &complications
  • 10. CCLLIINNIICCAALL PPIICCTTUURREE 33××33 Mucosal bleeding Visual changes Neurological symptoms Cardiopulmonary symptoms Renal symptoms &Dermatological . Constitutional sym
  • 11. Mucosal bleeding • SSppoonnttaanneeoouuss gguumm bblleeeeddiinngg • EEppiissttaaxxiiss • RReeccttaall bblleeeeddiinngg • MMeennoorrrrhhaaggiiaa • PPeerrssiisstteenntt bblleeeeddiinngg aafftteerr mmiinnoorr procedures
  • 12.
  • 13. VViissuuaall cchhaannggeess rraannggee ffrroomm bblluurrrreedd vviissiioonn ttoo vviissiioonn lloossss.. Change in visual acuity: (BlurringDiplopiaVisual loss) Characteristic “link-sausage effect” on funduscopy Alternating bulges and constrictions within the retinal veins Retinal hemorrhage, detachment Exudate, microaneurysm formation Papilledema
  • 14.
  • 15. NNeeuurroollooggiiccaall ssyymmppttoommss FFrreeqquueenntt&&VVaarriiaabbllee • VVeerrttiiggoo • HHeeaarriinngg lloossss • PPaarreesstthheessiiaass • AAttaaxxiiaa • HHeeaaddaacchheess • SSeeiizzuurreess • SSoommnnoolleennccee pprrooggrreessssiinngg ttoo ssttuuppoorr aanndd ccoommaa
  • 16.
  • 17. CCaarrddiiooppuullmmoonnaarryy ssyymmppttoommss CCoonnssttiittuuttiioonnaall ssyymmppttoommss Dyspne Dysrhythmias Hypoxia Heart failure Fatigue Lethergy Anorexia
  • 18. DDeerrmmaattoollooggiiccaall RReennaall ssyymmppttoommss RRaayynnaauudd pphheennoommeennoonnoo LLiivveeddoo rreettiiccuullaarriiss PPaallppaabbllee ppuurrppuurraa EErruuppttiivvee ssppiiddeerr nneevvuuss––lliikkee DDiiggiittaall iinnffaarrccttss PPeerriipphheerraall ggaannggrreennee Nephritic or nephrotic syndrome hematuria Sterile pyuria
  • 19. PPeeaarrll ttoo bbee hhiigghhlliigghhtteedd To early diagnose a patient with HVS you should have high index of suspicion for it in any patient with unexplained MMuuccoossaall bblleeeeddiinngg ((NNoossee&&gguumm)) NNeeuurroollooggiiccaall CCOO …………HHeeaaddaacchhee BBlluurrrriinngg ff vviissiioonn DDyyssppnneeaa EEssppeecciiaallllyy tthhoossee wwiitthh uunnddeerrllyyiinngg bblloooodd ddiisseeaassee
  • 20. PPhhyyssiiccaall EExxaammeennaattiioonn Related to the cause : spleanomegaly &flushed face in PRV :Related to organ affected Bruises, epistaxis, or gum bleeding may be noted Ophthalmic examination :decreased visual acuity,dilated retinal veins, "sausage-linked"of the retinal veins, or retinal Hge , Neurologic examination may reveal various abnormalities including diminished mental status, confusion, ataxia, or nystagmus Cardiopulmonary examination may reveal signs of CHF with (volume overload (rales, LLO,CNV, and hypoxia
  • 22. HHiissttoorryy && CClliinniiccaall eexxaammeennaattiioonn Lab. Investigations Imaging
  • 23. HHiissttoorryy && CClliinniiccaall eexxaammeennaattiioonn Put in mind any Pt presented with especially if unexplained and the 3×3 Pt is suspected to have underlying hematological disease
  • 24. . Put in mind lab • Comment on the • P t samples if it • is difficult in • manipulations • and separation •
  • 25. Lab. clues CBC with blood film Globulin gap Measurement of serum viscosity Metabolic panel and Electrolyte Urine analysis Coagulation profile Important markers SPEP and SPIF
  • 26. CBC with blood film Erythrocytosis Leukemia Thrombocytosis N N anemia with rouleaux formation
  • 27. Globulin gap Consider measuring total protein ,TP) and albumin) ; as in the paraproteinemias a globulin gap TP – albumin = 4 or greater) may ) exist
  • 28. Measurement of serum viscosity Ostwald viscosimeter Normal range for the serum viscosity relative .to water is 1.4–1.8 Minimal viscosity at which symptoms develop is 4.0 centipoise (.cp )
  • 29. :Metabolic panel and Electrolyte Renal dysfunction is commonly noted in HVS Hypercalcemia and pseudohyponatremia in MM
  • 30. : Urine analysis Proteinuria Hematuria Sterile pyuria BJP Protein Electrophoresis
  • 31. Coagulation profile For any coagulopathy workup …PT,PTT,BT)
  • 32. Important markers LDH, B2 Microglobulins, serum vit B 12 Uric acid and ALP.
  • 34. Tailor additional workup • according • to patient presentation
  • 35. Imaging Bone survey Us abdomen and Pelvis CT &MRI brain CXR ECHO cardiography
  • 37. Pre-hospital Stabilization Therapy A,B,C Care with keeping IV fluid axis(Rehydrate with 0.9% NS IV fluid.) Avoid blood transfusion ?
  • 38. Emergency Departement Treatment plasmapheresis Hydration Early apheresis and phlebotomy Standard therapies For CHF, bleeding… platletpheresis leukapheresis Erythrocytosis
  • 39. Be Wise enough☺ Be careful in p RBCs transfusion even when needed (v.slow) Diuretic may worsen the condition TLS can occure easily with leukapheresis IDA can occure from repeated venesiction and lead to microcytosis with more HV Phlebotomy with hydration (in non anemic Pt)may be clue Till Apheresis is available
  • 40. Note that the definitive treatment of HVS is treatment of the underlying disorder eg, chemotherapy). If) the underlying disease process is left untreated . the HV will recur,
  • 41. Prognosis :It depends on Severity of the complications, The underlying cause Response of the appropriate definitive treatment.
  • 42. NNooww…….. MMeennttaall bbrreeaakk !! ......إإننسسوواا ......إإننسسوواا ......إإننسسوواا
  • 43.
  • 44. HVS is a clinical entity that can be fatal but can also be easily treated if early diagnosed For health care supervisors to diagnose HVS they must Have high index of suspicion towards Unexplained mucosal bleeding dyspnea and coma Blood film is very important and may be clue for many case Keep in mind lab. Comments on your patient samples .If it is repeatedly condense and block lab. Machines Be wise enough in judging patients with HVS even in Treating current problem you may worsen the matter
  • 45. SSuurree yyoouu aarree ttiirreedd...... EEnnoouugghh ......!!PPlleeeeeeeeeezz