Download - Hyperviscosity syndrome
Hyperviscosity Syndrome
ByMohamed AbdElmotaal Safa
Ass. Lecturer of Internal MedicineFaculty of Medicine
Tanta University
Physiological backgroundPhysiological background
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
(85 to 90%(
CELLS
(10 to 15%(
RBCs WBCsPlat.
Polycyathemia
Leukemia
Thrombocytosis
Polyclonal
Monoclonal
HV of Blood HV of Blood
MMMM
WMWM
MGUSMGUS
1ry Amyloidosis1ry Amyloidosis
Heavy chain Heavy chain
diseasedisease
InfectionsInfections
TumorsTumors
Liver failureLiver failure
Collagen diseaseCollagen disease
SarcoidosisSarcoidosis
PathophysiologyPathophysiology
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
AGE
SEX
MORTALITY
More in old age
More in males
Related to
cause &complications
Data are non conclu
sive
due to
lake of
info
rmatio
ns
CLINICAL PICTURE 3×3CLINICAL PICTURE 3×3
Mucosal bleeding
Neurological symptoms
Visual changes
Cardiopulmonary
symptoms
Dermatological &
Constitutional sym.
Renal symptoms
Mucosal bleeding
• Spontaneous gum bleedingSpontaneous gum bleeding• EpistaxisEpistaxis• Rectal bleedingRectal bleeding• MenorrhagiaMenorrhagia• Persistent bleeding after minorPersistent bleeding after minor procedures
Visual changesVisual changes
range from blurred vision to vision lossrange from blurred vision to vision loss..Change in visual acuity: (BlurringDiplopiaVisual loss)Characteristic “link-sausage effect” on funduscopyAlternating bulges and constrictions within the retinal veinsRetinal hemorrhage, detachmentExudate, microaneurysm formationPapilledema
Neurological symptomsNeurological symptoms
• VertigoVertigo• Hearing lossHearing loss• ParesthesiasParesthesias• AtaxiaAtaxia• HeadachesHeadaches• SeizuresSeizures• Somnolence progressing to stupor and Somnolence progressing to stupor and
comacoma
Frequent&VariableFrequent&Variable
CardiopulmonaryCardiopulmonary
symptoms symptoms
ConstitutionalConstitutional
symptomssymptoms
DyspneDysrhythmiasHypoxia Heart failure
FatigueLethergyAnorexia
DermatologicalDermatological
Renal symptomsRenal symptoms
Raynaud phenomenonoRaynaud phenomenono
Livedo reticularisLivedo reticularis
Palpable purpuraPalpable purpura
Eruptive spider nevus–like Eruptive spider nevus–like
Digital infarctsDigital infarcts
Peripheral gangrenePeripheral gangrene
Nephritic or nephrotic syndromehematuriaSterile pyuria
Pearl to be Pearl to be
highlightedhighlighted
To early diagnose a patient
with HVS you should have
high index of suspicion for
it
in any patient with
unexplained
Mucosal bleedingMucosal bleeding
))Nose&gumNose&gum((
Neurological CONeurological CO
HeadacheHeadache…………
DyspneaDyspnea
Especially those with underlying blood diseaseEspecially those with underlying blood disease
Blurring f visionBlurring f vision
Physical ExamenationPhysical Examenation
Related to the cause : spleanomegaly &flushed face in PRV
Related to organ affected:Bruises, epistaxis, or gum bleeding may be notedOphthalmic examination :decreased visual acuity,dilated retinal
veins, "sausage-linked"of the retinal veins, or retinal HgeNeurologic 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
History & Clinical History & Clinical
examenationexamenation
Imaging
Lab. Investigations
History & Clinical History & Clinical
examenationexamenation
Put in mind any Pt presented with 3×3 especially if unexplained and the
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
CBC with blood filmGlobulin gapMeasurement of serum viscosity Metabolic panel and ElectrolyteUrine analysisCoagulation profileImportant markersSPEP and SPIF
Lab. clues
ErythrocytosisLeukemiaThrombocytosisN N anemia with
rouleaux formation
CBC with blood film
Consider measuring total protein )TP) and albumin,
as in the paraproteinemias; a globulin gap
)TP – albumin = 4 or greater) may exist
Globulin gap
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(.
Measurement of serum viscosity Ostwald viscosimeter
Renal dysfunction is commonly noted in HVS Hypercalcemia and pseudohyponatremia in MM
Metabolic panel and Electrolyte:
ProteinuriaHematuriaSterile pyuriaBJP
Protein Electrophoresis
Urine analysis:
For any coagulopathy workup
)PT,PTT,BT…
Coagulation profile
LDH, B2 Microglobulins, serum vit B 12Uric acid and ALP.
Important markers
SPEP and SPIF
•Tailor additional workup according• to patient presentation
Bone surveyUs abdomen and PelvisCT &MRI brain CXRECHO cardiography
Imaging
TREATEMENT
A,B,C Care with keeping IV fluid axis(Rehydrate with 0.9% NS IV fluid.)Avoid blood transfusion ?
Pre-hospital Stabilization Therapy
Hydration Early apheresis and phlebotomy Standard therapiesFor CHF, bleeding…
Emergency Departement Treatment
plasmapheresis
platletpheresis
leukapheresis
Erythrocytosis
Be careful in p RBCs transfusion even when needed (v.slow)Diuretic may worsen the condition TLS can occure easily with leukapheresisIDA can occure from repeated venesiction and lead to microcytosis with more HVPhlebotomy with hydration (in non anemic Pt)may be clue Till Apheresis is available
Be Wise enough☺
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.
It depends on : Severity of the complications,
The underlying cause Response of the appropriate definitive treatment.
Prognosis
......إنسواإنسوا
......إنسواإنسوا
......إنسواإنسوا
Now…. Mental break !Now…. Mental break !
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 mustHave high index of suspicion towards Unexplained
mucosal bleeding dyspnea and comaBlood film is very important and may be clue for many
case
Keep in mind lab. Comments on your patient samplesIf 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
Sure you are Sure you are
tired...tired...
EnoughEnough
PleeeeezPleeeeez...!...!