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SPLENOMEGALY
By Dr Bashir Ahmed DarBy Dr Bashir Ahmed Dar
Associate Professor MedicineAssociate Professor Medicine
Chinkipora Sopore KashmirChinkipora Sopore Kashmir
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Slight enlargement just palpable (less
than 5 cm) Infections acute,subacute and chronicInfections acute,subacute and chronic
SLESLE
Rheumatoid arthritisRheumatoid arthritis
AmyloidosisAmyloidosis
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Moderate enlargement (to
umbilicus) LymphomasLymphomas
LeukaemiasLeukaemias
Congestive splenomegalyCongestive splenomegaly Haemolytic anaemiasHaemolytic anaemias
Polycythaemia VeraPolycythaemia Vera
ITPITP Myelodysplastic disordersMyelodysplastic disorders
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Marked enlargement (more than
8 cm or below umbilicus) MyelofibrosisMyelofibrosis
Hairy cell leukaemiaHairy cell leukaemia
Tropical splenomegalyTropical splenomegaly Kala azarKala azar
Thalassaemia majorThalassaemia major
Gauchers diseaseGauchers disease Feltys syndromeFeltys syndrome
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Infective causes of splenomegaly
Acute causesAcute causes
Infectious mononucleosisInfectious mononucleosis
TyphoidTyphoid BrucellosisBrucellosis
Infective hepatitisInfective hepatitis
ToxoplasmosisToxoplasmosis TyphusTyphus
SepticaemiaSepticaemia
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Infective causes of splenomegaly
Subacute and chronic causesSubacute and chronic causes
Bacterial endocarditisBacterial endocarditis
TuberculosisTuberculosis
BrucellosisBrucellosis SyphilisSyphilis
HistoplasmosisHistoplasmosis
MalariaMalaria
Kala azarKala azar
HydatidHydatid
TrypanosomiasisTrypanosomiasis
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Massive splenomegaly
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Rheumatoid arthritis (Feltys
syndrome)
Comprises ofComprises of
Arthritis,Splenomegaly,LeucopeniaArthritis,Splenomegaly,Leucopenia
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Features of Feltys syndrome
Arthritis,splenomegaly,leucopeniaArthritis,splenomegaly,leucopenia
Extra-Articular ManifestationsExtra-Articular Manifestations
rheumatoid nodules (76%)rheumatoid nodules (76%)
weight loss (68%)weight loss (68%) Sjogren's Syndrome (56%)Sjogren's Syndrome (56%)
lymphadenopathy (34%)lymphadenopathy (34%)
leg ulcers (25%)leg ulcers (25%)
pleuritis (19%)pleuritis (19%)
skin pigmentation (17%)skin pigmentation (17%) neuropathy (peripheral) -17%neuropathy (peripheral) -17%
episcleritis (8%)episcleritis (8%)
others: pericarditisothers: pericarditis
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Investigations Felty's syndrome
high Rf titre in 98%high Rf titre in 98%
ANA in 67%ANA in 67%
elevated ESR, immunoglobulins, circulatingelevated ESR, immunoglobulins, circulatingimmune complexesimmune complexes
positive LE cell test in 33%positive LE cell test in 33%
decreased complement levelsdecreased complement levels elevated transaminases and alkalineelevated transaminases and alkaline
phosphatase in 25-50%phosphatase in 25-50%
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Tropical Splenomegaly
In tropical areas massive splenomegalyIn tropical areas massive splenomegaly
often seenoften seen
Areas like Uganda,Nigeria,new guinea andAreas like Uganda,Nigeria,new guinea and
other parts of Africaother parts of Africa
The evidence suggests a relationshipThe evidence suggests a relationship
between malaria and tropical splenomegalybetween malaria and tropical splenomegaly Rarely occurs in malarial free areasRarely occurs in malarial free areas
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Tropical Splenomegaly
Malarial parasite are not routinely seen in bloodMalarial parasite are not routinely seen in bloodfilms of ptsfilms of pts
The disorder usually presents in young adult lifeThe disorder usually presents in young adult lifebut may occur in childrenbut may occur in children
The diagnosis is usually made by exclusion ofThe diagnosis is usually made by exclusion ofother causesother causes
There may also be 10 fold increase polyclonal igMThere may also be 10 fold increase polyclonal igMconcentration in serum of which small portionconcentration in serum of which small portionrepresents malarial antibodies liver may also showrepresents malarial antibodies liver may also showlymphocytic infiltrationlymphocytic infiltration
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Kala azar (leishmaniasis)
Visceral leishmaniasisVisceral leishmaniasis
Cutaneous leishmaniasisCutaneous leishmaniasis
Mucocutaneous leishmaniasis (espundia)Mucocutaneous leishmaniasis (espundia)
Diffuse Cutaneous leishmaniasisDiffuse Cutaneous leishmaniasis
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kala azar, black fever, sandflydisease, Dum-Dum fever and
espundia.
SYNONYMS
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in the familyTrypanosomatidae
In the genus Leishmania
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EstimatesAll over world except
Not found in South-east Asia
350 million people at risk12 million people infected / year
There are 59,000 deaths / year
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Incubation 1 week to monthsIncubation 1 week to months
Having (many reservoirs)Having (many reservoirs)
No direct person to person transmissionNo direct person to person transmission Spontaneous healing can happen (cutaneousSpontaneous healing can happen (cutaneous
form) in months to yearsform) in months to years
No vaccineNo vaccine
Some Facts
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LEISHMANIASIS or KALA-AZAR
It is the result of the infection with oneIt is the result of the infection with oneof the species of protozoa (leishmania).of the species of protozoa (leishmania).
Conveyed by:Conveyed by:
Sandflies (Phlebotomus).Sandflies (Phlebotomus).
Visceral LeishmaniasisVisceral Leishmaniasis: L. donovani: L. donovani
MucocutaneousMucocutaneous : L. Braziliensis: L. Braziliensis
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L i h i
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Have specific reservoirs
Leishmania
Parasites
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Rodents
Sloths
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VectorsPhlebotomine Sand Flies
20 t 40 d
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20 to 40 days
30to70eggs
hatch
1to2weeks
4 instars
diapauses in 4th instar
pupald
evelop
ment
5-10
day
s
adults crepuscular
and nocturnal
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Opossum
Armadillo
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Visceral LeishmaniasisVisceral Leishmaniasis::
L. donovaniL. donovaniMucocutaneousMucocutaneous
L. BraziliensisL. Braziliensis
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Cutaneous : L. tropica majorL. tropica minor
New World : L. BrazilienL. Mexicana
Oldworld
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PATHOLOGY
L. donovani parasitizes theL. donovani parasitizes the reticu. endoth.reticu. endoth.cellscells
Great proliferation of macrophageGreat proliferation of macrophage
Cells result:Cells result: Liver spleenLiver spleen enlarg.enlarg.
The red bone marrow extendThe red bone marrow extend..
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Leishmania: an obligate intracellular
protozoan parasite (2-6 m in diameter)
parasi
tophor
ous
vacuol
esof
macro
phages
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Promastigotes in Sand Fly gut
and in Culture Media
(about1
5-30
m
by2
-3m
),
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Amastigote in macrophage
TheThe
amostigotes ofamostigotes of
differentdifferent
species arespecies arevery similar onvery similar on
lightlight
microscopy.microscopy.
CLINICAL PICTURE
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CLINICAL PICTURE
early stages is not easy for diagnose.early stages is not easy for diagnose.
There is no constantThere is no constant physical signs.physical signs.
Changes in the blood pictureChanges in the blood pictureparticularlyparticularly Leucopenia.Leucopenia.
Outstanding physical sign is theOutstanding physical sign is theenlargement of the spleen 3 cm. aenlargement of the spleen 3 cm. a month.month.
LiLiver : enlarged spleen + liver are: enlarged spleen + liver are
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LiverLiver : enlarged spleen + liver are: enlarged spleen + liver are
neither tender nor painful.neither tender nor painful.
SometimesSometimes: Jaundice = prognost.: Jaundice = prognost.
SignificanceSignificance
EnlargedEnlarged : Lymph node, could be: Lymph node, could be
but its not a feature of thebut its not a feature of the
disease.disease.
WastingWasting : Emaciated pat with a: Emaciated pat with a
protuberantprotuberant
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Abdomen ( liver + spleenAbdomen ( liver + spleenenlarged)enlarged)
FeverFever : Without subjective symptoms: Without subjective symptomsof fever no delirium.of fever no delirium.
SometimesSometimes: there is no fever: there is no fever
SkinSkin : dry, rough. The natural: dry, rough. The naturalpigmentation of the skin overpigmentation of the skin overthe bone and around thethe bone and around the
mouth is deepened. (Kalamouth is deepened. (Kala
azar??)azar??)
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Clinical Forms of Leishmaniasis
Cutaneous
Mucocutaneous
DiffuseCutaneous
Visceral
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Cutaneous form
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Wet lesion by L.major
L. majoroccurs most commonlyin rural areas, causing moist,
ulcerative lesions which may beextensive and sometimes involvethe epithelium of lips and nose.
These are thecommonest types of
lesion caused by L.major.
Prominent rolled edgeof the lesions is the bestarea to demonstrate theparasites
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Lymphatic spread ofL.major
The ink marks indicate a lineof subcutaneous nodulesalong the lymphatic, passingproximally from the lesion on
the lower part of this mansarm.
The nodules usually resolvewithout complications whenthe primary lesin heals with
or without specific therapy.
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Clinical Forms
of
Leishmaniasis
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Simple dry lesion by L.tropica
dry, usually self-healing lesions,
Generally single
Urban area inNorth Africa andthe Middle East tothe former USSR,Afghanistan,western states ofIndia
The lesions
frequently containvery largenumbers ofparasites.
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Leishmaniasis recidivans
Infection with L. tropica maybecome very chronic, with ahyperallergic reaction leading tolupus-like lesions such as seen in
this child. Amastigotes may be very difficult to
find in the lesions.
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Mucocutaneous lesion by L.aethiopica
In addition to simplecutaneous lesionsdue to infection withL. aethiopica, otherforms are seen inthe Ethiopian
highlands whererock hyraxes are thereservoirs. It has notyet beenascertained whetherthe mucocutaneous
condition seen hereis due to this oranother species ofLeishmania.
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Chicleros ulcer by L.mexicana
Forest workersForest workers
collecting gum fromcollecting gum from
wild chicle treeswild chicle trees
commonly sleepcommonly sleep
near the forest floornear the forest floorand are bitten onand are bitten on
exposed parts of theexposed parts of the
head by vectors.head by vectors.
Ulcers leading toUlcers leading to
erosion of theerosion of theauricular cartilage.auricular cartilage.
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Lymphatic spread of
L. mexicana
As noted for L. major,lymphatic spread mayalso occur with NewWorld species.
This patient wasinfected with L.guyanensis the agentof Pian bois. In suchinfections the
lymphatic nodules mayulcerate.
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Pharyngeal involvement
Ulceration often extends to the pharynx and soft palate,and the first symptoms may be related to tissue destructionin this area.
This man had the scar of a large ulcer which had apparentlyhealed on his leg some 30 years before.
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PKDLPost Kalazar Dermal Leishmaniasis
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Visceral form
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Clinical picture of kala-azar in Kenya
Increasingenlargement ofthe spleen andliver is acharacteristicfeature while, indark-complexionedsubjects,deepening skinpigmentation is
seen-hence thesynonym kala-azar, the blacksickness.
A generalized lymphadenopathy is common in Africankala-azar; the parasite in this area is considered to be inthe L. donovani complex.
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Diagnosis
Demonstration of Parasite from aspirateDemonstration of Parasite from aspirate
- lymph node- lymph node
; inguinal LN, sensitivity 60%; inguinal LN, sensitivity 60%
- bone marrow- bone marrow
; iliac crest, sensitivity 70%; iliac crest, sensitivity 70%- spleen; sensitivity 95~99%- spleen; sensitivity 95~99%
- liver- liver
PB smear (HIV infected patient)PB smear (HIV infected patient)
SerologySerology- a- avoid the necessity for the more invasive procedurevoid the necessity for the more invasive procedure- direct agglutination test- direct agglutination test
PCRPCR
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Iliac crest aspiration of bone marrow
The most directmeans ofdiagnosis of kala-azar is thedetection ofamastigotes inbone-marrow,spleen or blood;the organisms arerecognized indried smears of
material stainedwith aRomanowsky stainby theircharacteristicmorphology.
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L. infantum in macrophage from bone marrow
While typically found in macrophages as shown here,While typically found in macrophages as shown here,isolated extracellular amastigotes from disrupted hostisolated extracellular amastigotes from disrupted host
cells are commonly seen in such preparations.cells are commonly seen in such preparations.
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Amastigotes in macrophage fromskin
The diagnosis isconfirmed bydemonstraingamastigotes insmear made
from thecutaneouslesions.
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Punch biopsy
A piece of tissuemay be removedunder localanesthetic with adisposable skin
punch forhistology, cultureand the directdemonstration ofamastigotes.
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Parasitised macrophage in skinsection
Manyparasitisedmacrophagescan be seen inthis section
from an acutelesion caused byL. major.
DIAGNOSIS
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DIAGNOSIS1.1. Needle aspirationNeedle aspiration
Bone sternum liver, spleenBone sternum liver, spleenHistology cultureHistology culture
L. donovani bodyL. donovani body
2.2. a.a. LeucopeniaLeucopenia:: Neutropenia Neutropenia relative mononucleosis.relative mononucleosis.
b.b. Progressive fall with the red cellProgressive fall with the red cell
countcount
Formalin gel (aldehydeForm
alin gel (aldehyde)
)
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g ( yg ( y ))
2 drops formalin + 2 ml serum2 drops formalin + 2 ml serum
After 20 min, white ringAfter 20 min, white ring
5.5. Complement fixation and fluorescentComplement fixation and fluorescent
False positive trypanosomal infectionFalse positive trypanosomal infection
The complement fixationThe complement fixation early positive early positive
negative after cure. Sometimes + or lung. Othernegative after cure. Sometimes + or lung. Other
diseases.diseases.
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6.6. Fluorescent antibodyFluorescent antibody
IV + V:IV + V: In trypanosomal infectionIn trypanosomal infection
Skin test (Montenegro)Skin test(Montenegro)
Delayed hypersensitivity reactionDelayed hypersensitivity reaction
0.2 ml. suspension0.2 ml. suspension
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TREATMENT AntimonialAntimonial
i.i. Urea, stibamine, pentavalen + antmonyiaUrea, stibamine, pentavalen + antmonyia
I.V. daily or every 2 daysI.V. daily or every 2 days
6 10 dose6 10 dose
First 100 mg then 200 then 250First 100 mg then 200 then 250
Total dose 2 5 g. adultTotal dose 2 5 g. adult
Side EffectSide Effect:: Nausea, vomiting, joint pain,Nausea, vomiting, joint pain,
Abdominal pain, diarrheaAbdominal pain, diarrhea
C t i di tiContraindication: Li d kidLiver and kidney
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ContraindicationContraindication:: Liver and kidneyLiver and kidney
failurefailure
ii.ii. Sodium stibogluconate (PentostamSodium stibogluconate (Pentostam)) I.M. 600 mg total daily for 6-10 daysI.M. 600 mg total daily for 6-10 days
Repeated after 14 days; if neededRepeated after 14 days; if needed
Side effectSide effect::Anaphylactic shockAnaphylactic shock
7.7. Diamidiem Diamidiem
Pentamidine isothionatePentamidine isothionate
Dose 3 -4 mg / kg / BW total 300 mgDose 3 -4 mg / kg / BW total 300 mg
Side effectSide effect:: HypoglycemiaHypoglycemia
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Treatment
Sodium stibogluconateSodium stibogluconate
Pentavalent antimony (SbPentavalent antimony (Sb+5+5))
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demonstration of amastigotes(L. donovani bodies)demonstration of amastigotes(L. donovani bodies)
intracellular form is usually 2-4 micron consists ofintracellular form is usually 2-4 micron consists ofnucleus and is rod like with a homogeneous massnucleus and is rod like with a homogeneous mass
of cytoplasm,while the extracelluar or culture formof cytoplasm,while the extracelluar or culture form
is14-20 microns in length 1.5 to 3.5 breadthis14-20 microns in length 1.5 to 3.5 breadth
L. donovani bodyL. donovani body
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Myelofibrosis or myelosclerosis
Fibrosis and collagen formation in marrowFibrosis and collagen formation in marrow
Primary developing in polycythemia veraPrimary developing in polycythemia vera
Secondary in TB,secondarySecondary in TB,secondarycarcinoma,hodgkins disease,leukaemia,andcarcinoma,hodgkins disease,leukaemia,and
variety of other conditions.variety of other conditions.
Myeloid metaplasia ,spleenomegalyMyeloid metaplasia ,spleenomegalyhepatomaegaly etc etchepatomaegaly etc etc
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GAUCHERS DISEASE
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History
Discovered by Philippe Gaucher, a medicalDiscovered by Philippe Gaucher, a medical
student in Paris, in 1882.student in Paris, in 1882.
He was studying a woman with an enlargedHe was studying a woman with an enlarged
spleenspleen
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DefinitionDefinition
Gaucher's (go-SHAYZ) disease occursGaucher's (go-SHAYZ) disease occurs
when certain harmful fatty substanceswhen certain harmful fatty substancesbuild to excessive levels in your liver,build to excessive levels in your liver,
spleen, lungs, bone marrow and, lessspleen, lungs, bone marrow and, less
commonly, your brain. Thiscommonly, your brain. This
accumulation of fatty material in tissuesaccumulation of fatty material in tissuesinterferes with the normal functioning ofinterferes with the normal functioning of
organs, and may cause organorgans, and may cause organ
enlargement and bone pain.enlargement and bone pain.
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Gaucher's disease results from anGaucher's disease results from an
enzyme deficiency, and sometimes theenzyme deficiency, and sometimes the
term "glucocerebrosidase deficiency" isterm "glucocerebrosidase deficiency" is
used to describe this condition.used to describe this condition.
Gaucher's disease is most common inGaucher's disease is most common in
Eastern and Central EuropeanEastern and Central European
(Ashkenazi) Jews. It can occur at any(Ashkenazi) Jews. It can occur at anyage in life, and affects males andage in life, and affects males and
females approximately equally.females approximately equally.
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What Is It?
Gauchers DiseaseGauchers Disease- rare inherited metabolic- rare inherited metabolicdisease or disorder ; due deficiency or lack of andisease or disorder ; due deficiency or lack of anenzyme calledenzyme called GlucocereborsidaseGlucocereborsidase
Results in an accumulation ofResults in an accumulation ofglucocerebrosideglucocerebrosidewithin cells in various body tissues ( spleen, liver,within cells in various body tissues ( spleen, liver,
bone marrow, and skeleton)bone marrow, and skeleton)
Severity of the disease can vary and thus diseaseSeverity of the disease can vary and thus diseasedivided into following typesdivided into following types
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TYPE 1
Most common and can begin at any ageMost common and can begin at any age
1 in 10,0001 in 10,000 Patients are bruised very easilyPatients are bruised very easily
Fatigued due to anemiaFatigued due to anemia
Lung and kidney injuriesLung and kidney injuries
Weakening of the skeletonWeakening of the skeleton
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TYPE 1 (continued)
Victims have a shortened life-spanVictims have a shortened life-span
Usually die from clots and pneumoniaUsually die from clots and pneumonia
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TYPE 2
Rarest of all the typesRarest of all the types
Appears during the first few months of lifeAppears during the first few months of lifein a babyin a baby
Great brain damage mental retardationGreat brain damage mental retardation
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TYPE 2 (continued)
Loss of muscle controlLoss of muscle control
Enlargement of liver and spleenEnlargement of liver and spleen
Nervous system fails to function wellNervous system fails to function well
Patients usually die by age 2Patients usually die by age 2
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TYPE 3
Begins in childhoodBegins in childhood
Liver and spleen enlargementLiver and spleen enlargement
Causes bone marrow and damages theCauses bone marrow and damages thecentral nervous systemcentral nervous system
Mental retardation is quite commonMental retardation is quite common
Usually die around the ages 15-30Usually die around the ages 15-30
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Symptoms (continued)
Muscle weaknessMuscle weakness
Poor coordinationPoor coordination
SeizuresSeizures
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How Do You Get It?
Acquired if both parents of the disease areAcquired if both parents of the disease are
carrierscarriers
A victim receives an abnormal form of theA victim receives an abnormal form of thegenes- agenes- a Gaucher GeneGaucher Gene from both parentsfrom both parents
CarrierCarrier- person with one normal gene and- person with one normal gene and
one Gaucher Gene ( a carrier will not showone Gaucher Gene ( a carrier will not showsigns of the disease)signs of the disease)
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If 2 Carriers Have Children
There is a one in four chance of aThere is a one in four chance of a
child inheriting the diseasechild inheriting the disease
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Who Has It?
10,000 to 20,000 Americans10,000 to 20,000 Americans
High rate found in the Ahkenazi JewishHigh rate found in the Ahkenazi Jewish
population-1 out of 500-1000 birthspopulation-1 out of 500-1000 births Types 2 & 3 are found in 1 out of 50,000-Types 2 & 3 are found in 1 out of 50,000-
100,000 births100,000 births
Type 3-mainly found in people of northernType 3-mainly found in people of northernSwedish AncestrySwedish Ancestry
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Prevention
No real preventionNo real prevention
Genetic Counseling is recommended forGenetic Counseling is recommended for
parents with a family history of the diseaseparents with a family history of the disease
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Treatments
NoNo CURECURE
Enzyme replacement therapy-injections ofEnzyme replacement therapy-injections of
the enzymethe enzyme Result: decrease liver and spleen sizeResult: decrease liver and spleen size
reduce skeletal abnormalitiesreduce skeletal abnormalities
restores normal growth &restores normal growth &developmentdevelopment
restores well being of the patientrestores well being of the patient
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Hairy cell leukaemia
Hairy cell leukaemia is an uncommonHairy cell leukaemia is an uncommon
disorder of middle and late adult life.disorder of middle and late adult life.
Characterized by the presence in boneCharacterized by the presence in bonemarrow,spleen,and peripheral blood ofmarrow,spleen,and peripheral blood of
abnormal mononuclear cells with hairyabnormal mononuclear cells with hairy
cytoplasmic projections and best detectedcytoplasmic projections and best detectedby phase contrast microscopy.by phase contrast microscopy.
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Males affected more than femalesMales affected more than females
Marked by splenomegaly , howeverMarked by splenomegaly , however
lymphadenopathy is unusuallymphadenopathy is unusual Splenectomy is usually regarded asSplenectomy is usually regarded as
treatment of choicetreatment of choice
Long term use of injections of alphaLong term use of injections of alphainterferon cause regression of hairy cellsinterferon cause regression of hairy cells
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Hairy cell leukemia is actually a mature BHairy cell leukemia is actually a mature B
cell neoplasm. It is usually classified as acell neoplasm. It is usually classified as a
sub-type of chronic lymphoid leukemia forsub-type of chronic lymphoid leukemia forconvenience. It is uncommon, representingconvenience. It is uncommon, representing
about 2% of all leukemias, or less than aabout 2% of all leukemias, or less than a
total of 2000 new cases diagnosed each yeartotal of 2000 new cases diagnosed each year
in the North America and Western Europein the North America and Western Europe
combined.combined.
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Originally known as histiocytic leukemia,Originally known as histiocytic leukemia,
malignant reticulosis, or lymphoidmalignant reticulosis, or lymphoid
myelofibrosis in publications dating back tomyelofibrosis in publications dating back tothe 1920s, this disease was formally namedthe 1920s, this disease was formally named
leukemic reticuloendotheliosisleukemic reticuloendotheliosis
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SymptomsSymptoms
In hairy cell leukemia, the broken "hairy cells"In hairy cell leukemia, the broken "hairy cells"build up in the bone marrow, which means that thebuild up in the bone marrow, which means that thebone marrow has difficulty producing enoughbone marrow has difficulty producing enoughnormal cells: white blood cells to fight infections,normal cells: white blood cells to fight infections,red blood cells to carry oxygen, and platelets tored blood cells to carry oxygen, and platelets to
stop bleeding. Consequently, patients usuallystop bleeding. Consequently, patients usuallypresent with infection, anemia-related fatigue,present with infection, anemia-related fatigue,and/or easy bleeding.and/or easy bleeding.
H t l i Di d C i
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Hematologic Disorders Causing
Massive Splenomegaly Polycythemia VeraPolycythemia Vera Multiple MyelomaMultiple Myeloma
POEMS SyndromePOEMS Syndrome
Waldenstrm's MacroglobulinemiaWaldenstrm's Macroglobulinemia
chronic lymphocytic leukemiachronic lymphocytic leukemia
non-Hodgkin lymphomanon-Hodgkin lymphoma
chronic myelocytic leukemiachronic myelocytic leukemia malaria (hyper-reactive malarialmalaria (hyper-reactive malarial
splenomegaly)splenomegaly)
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