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...!...!