Causes of Splenomegaly By Dr Bashir Ahmed Dar Chinkipora Sopore Kashmir Associate Professor Medicine

Dr.Bashir Ahmed Dar Chinkipora Sopore Kashmir India,Associate Prof of medicine presently working in malaysia is a keen teacher, educator and takes pride in his clinical and research accomplishments. His interests include publishing articles related to health issues.Email [email protected]

1. SPLENOMEGALY
By Dr Bashir Ahmed DarBy Dr Bashir Ahmed Dar
Associate Professor MedicineAssociate Professor Medicine
Chinkipora Sopore KashmirChinkipora Sopore Kashmir
Email [email protected] [email protected]

2. Slight enlargement just palpable (less
than 5 cm)
Infections –acute,subacute and chronicInfections –acute,subacute and chronic
SLESLE
Rheumatoid arthritisRheumatoid arthritis
AmyloidosisAmyloidosis

3. Moderate enlargement (to
umbilicus)
LymphomasLymphomas
LeukaemiasLeukaemias
Congestive splenomegalyCongestive splenomegaly
Haemolytic anaemiasHaemolytic anaemias
Polycythaemia VeraPolycythaemia Vera
ITPITP
Myelodysplastic disordersMyelodysplastic disorders

4. Marked enlargement (more than
8 cm or below umbilicus)
MyelofibrosisMyelofibrosis
Hairy cell leukaemiaHairy cell leukaemia
Tropical splenomegalyTropical splenomegaly
Kala azarKala azar
Thalassaemia majorThalassaemia major
Gaucher’s diseaseGaucher’s disease
Felty’s syndromeFelty’s syndrome

5. Infective causes of splenomegaly
Acute causesAcute causes
Infectious mononucleosisInfectious mononucleosis
TyphoidTyphoid
BrucellosisBrucellosis
Infective hepatitisInfective hepatitis
ToxoplasmosisToxoplasmosis
TyphusTyphus
SepticaemiaSepticaemia

6. Infective causes of splenomegaly
Subacute and chronic causesSubacute and chronic causes
Bacterial endocarditisBacterial endocarditis
TuberculosisTuberculosis
BrucellosisBrucellosis
SyphilisSyphilis
HistoplasmosisHistoplasmosis
MalariaMalaria
Kala azarKala azar
HydatidHydatid
TrypanosomiasisTrypanosomiasis

7. Massive splenomegaly

8. Rheumatoid arthritis (Felty’s
syndrome)
Comprises of Comprises of
Arthritis,Splenomegaly,LeucopeniaArthritis,Splenomegaly,Leucopenia

9. Features of Felty’s 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: pericarditis others: pericarditis

10. Investigations Felty's syndrome
high Rf titre in 98% high Rf titre in 98%
ANA in 67% ANA in 67%
elevated ESR, immunoglobulins, circulating elevated ESR, immunoglobulins, circulating
immune complexes immune complexes
positive LE cell test in 33% positive LE cell test in 33%
decreased complement levels decreased complement levels
elevated transaminases and alkaline elevated transaminases and alkaline
phosphatase in 25-50% phosphatase in 25-50%

11. Tropical Splenomegaly
In tropical areas massive splenomegaly In tropical areas massive splenomegaly
often seenoften seen
Areas like Uganda,Nigeria,new guinea and Areas like Uganda,Nigeria,new guinea and
other parts of Africaother parts of Africa
The evidence suggests a relationship The evidence suggests a relationship
between malaria and tropical splenomegalybetween malaria and tropical splenomegaly
Rarely occurs in malarial free areasRarely occurs in malarial free areas

12. Tropical Splenomegaly
Malarial parasite are not routinely seen in blood Malarial parasite are not routinely seen in blood
films of ptsfilms of pts
The disorder usually presents in young adult life The disorder usually presents in young adult life
but may occur in childrenbut may occur in children
The diagnosis is usually made by exclusion of The diagnosis is usually made by exclusion of
other causesother causes
There may also be 10 fold increase polyclonal igM There may also be 10 fold increase polyclonal igM
concentration in serum of which small portion concentration in serum of which small portion
represents malarial antibodies liver may also show represents malarial antibodies liver may also show
lymphocytic infiltrationlymphocytic infiltration

13. Kala azar (leishmaniasis)
Visceral leishmaniasis Visceral leishmaniasis
Cutaneous leishmaniasis Cutaneous leishmaniasis
Mucocutaneous leishmaniasis (espundia)Mucocutaneous leishmaniasis (espundia)
Diffuse Cutaneous leishmaniasisDiffuse Cutaneous leishmaniasis

14. kala azar, black fever, sandfly
disease, Dum-Dum fever and
espundia.
SYNONYMS

15. Protozoan
in the family Trypanosomatidae
In the genus Leishmania

16. Estimates
All over world except
Not found in South-east Asia
350 million people at risk
12 million people infected / year
There are 59,000 deaths / year

17. 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 (cutaneous Spontaneous healing can happen (cutaneous
form) in months to yearsform) in months to years
No vaccineNo vaccine
Some Facts

18. LEISHMANIASIS or KALA-AZAR
It is the result of the infection with one It is the result of the infection with one
of 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

20. Have specific reservoirs
Leishmania Parasites

21. Rodents
Sloths

22. Vectors
Phlebotomine Sand Flies

23. 20 to 40 days
30 to 70 eggs
hatch 1 to 2 weeks
4 instars
diapauses in 4
th
instar
pupal development
5-10 days adults crepuscular
and nocturnal

24. Opossum
Armadillo

26. Visceral LeishmaniasisVisceral Leishmaniasis::
 L. donovaniL. donovani
MucocutaneousMucocutaneous
 L. BraziliensisL. Braziliensis

27. Cutaneous : L. tropica major
L. tropicaminor
New World : L. Braziliensis
L. Mexicana
Old
world

28. PATHOLOGY
»»L. donovani parasitizes the L. donovani parasitizes the reticu. endoth. reticu. endoth.
cellscells
»»Great proliferation of macrophageGreat proliferation of macrophage
»»Cells result:Cells result:Liver – spleen Liver – spleen enlarg.enlarg.
»»The red bone marrow extendThe red bone marrow extend..
»

29. Leishmania: an obligate intracellular
protozoan parasite (2-6 µm in diameter)
parasitophorous
vacuoles of
macrophages

30. Promastigotes in Sand Fly gut
and in Culture Media
(about 15-30 µm
by 2-3 µm),

31. Amastigote in macrophage
The amostigotes The amostigotes
of different of different
species are very species are very
similar on light similar on light
microscopy.microscopy.

32. CLINICAL PICTURE
early stages is not easy for diagnose. early stages is not easy for diagnose.
There is no constant There is no constant physical signs.physical signs.
☺☺Changes in the blood picture Changes in the blood picture
particularly particularly Leucopenia.Leucopenia.
☺☺Outstanding physical sign is the Outstanding physical sign is the
enlargement of the spleen 3 cm. a enlargement of the spleen 3 cm. a month.month.

33. ●●LiverLiver : enlarged spleen + liver are : enlarged spleen + liver are
neither tender nor painful. neither tender nor painful.
● ● SometimesSometimes: Jaundice = prognost. : Jaundice = prognost.
Significance Significance
● ● Enlarged Enlarged : Lymph node, could be : Lymph node, could be
but its not a feature of the but its not a feature of the
disease. disease.
●●WastingWasting: Emaciated pat with a : Emaciated pat with a
protuberant protuberant

34. Abdomen ( liver + spleen Abdomen ( liver + spleen
enlarged) enlarged)
●●FeverFever: Without subjective symptoms : Without subjective symptoms
of fever – no delirium. of fever – no delirium.
● ● SometimesSometimes: there is no fever: there is no fever
● ● SkinSkin: dry, rough. The natural : dry, rough. The natural
pigmentation of the skin over pigmentation of the skin over
the bone and around the the bone and around the
mouth is deepened. (Kala mouth is deepened. (Kala
azar??)azar??)

35. Clinical Forms of Leishmaniasis
• Cutaneous
• Mucocutaneous
• Diffuse Cutaneous
• Visceral

36. Cutaneous form

37. Wet lesion by L. major
L. major occurs most commonly in
rural areas, causing moist, ulcerative
lesions which may be extensive and
sometimes involve the epithelium of
lips and nose.
These are the commonest
types of lesion caused by L.
major.
Prominent ‘rolled’ edge of
the lesions is the best area
to demonstrate the
parasites

38. Lymphatic spread of L. major
The ink marks indicate a line of
subcutaneous nodules along the
lymphatic, passing proximally
from the lesion on the lower part
of this man’s arm.
The nodules usually resolve
without complications when the
primary lesin heals with or
without specific therapy.

39. Clinical Forms
of
Leishmaniasis

40. Simple ‘dry’ lesion by L. tropica
dry, usually self-
healing lesions,
Generally single
Urban area in North
Africa and the Middle
East to the former
USSR, Afghanistan,
western states of
India
The lesions
frequently contain
very large numbers
of parasites.

41. Leishmaniasis recidivans
Infection with L. tropica may become
very chronic, with a hyperallergic
reaction leading to lupus-like lesions
such as seen in this child.
Amastigotes may be very difficult to find
in the lesions.

42. Mucocutaneous lesion by L. aethiopica
In addition to simple
cutaneous lesions due
to infection with L.
aethiopica, other forms
are seen in the
Ethiopian highlands
where rock hyraxes are
the reservoirs. It has
not yet been
ascertained whether
the mucocutaneous
condition seen here is
due to this or another
species of Leishmania.

43. Chiclero’s ulcer by L. mexicana
Forest workers Forest workers
collecting gum from collecting gum from
wild chicle trees wild chicle trees
commonly sleep near commonly sleep near
the forest floor and are the forest floor and are
bitten on exposed parts bitten on exposed parts
of the head by vectors.of the head by vectors.
Ulcers leading to Ulcers leading to
erosion of the auricular erosion of the auricular
cartilage.cartilage.

44. Lymphatic spread of
L. mexicana
As noted for L. major,
lymphatic spread may also
occur with New World
species.
This patient was infected
with L. guyanensis the
agent of ‘Pian bois’. In
such infections the
lymphatic nodules may
ulcerate.

45. Early lesion of Espundia
The lesions of The lesions of mucocutaneous mucocutaneous
leishmaniasisleishmaniasis due to infection with due to infection with
L. braziliensisL. braziliensis may first become may first become
evident as ulcers involving the evident as ulcers involving the
mucocutaneous junctions of the mucocutaneous junctions of the
mouth and nosemouth and nose
This condition frequently follows a This condition frequently follows a
primary cutaneous lesion which primary cutaneous lesion which
may have healed with or without may have healed with or without
specific therapy some years earlier.specific therapy some years earlier.

46. Pharyngeal involvement
Ulceration often extends to the pharynx and soft palate, and the
first symptoms may be related to tissue destruction in this area.
This man had the scar of a large ulcer which had apparently healed
on his leg some 30 years before.

48. PKDL
Post Kalazar Dermal Leishmaniasis

55. Visceral form

58. Clinical picture of kala-azar in Kenya
Increasing
enlargement of the
spleen and liver is a
characteristic feature
while, in dark-
complexioned
subjects, deepening
skin pigmentation is
seen-hence the
synonym kala-azar,
the ‘black sickness’.
A generalized lymphadenopathy is common in African kala-azar;
the parasite in this area is considered to be in the L. donovani
complex.

60. 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

61. Iliac crest aspiration of bone marrow
The most direct
means of diagnosis
of kala-azar is the
detection of
amastigotes in bone-
marrow, spleen or
blood; the organisms
are recognized in
dried smears of
material stained with
a Romanowsky stain
by their characteristic
morphology.

62. L. infantum in macrophage from bone marrow
While typically found in macrophages as shown here, isolated While typically found in macrophages as shown here, isolated
extracellular amastigotes from disrupted host cells are extracellular amastigotes from disrupted host cells are
commonly seen in such preparations.commonly seen in such preparations.

63. Amastigotes in macrophage from skin
The diagnosis is
confirmed by
demonstraing
amastigotes in
smear made from
the cutaneous
lesions.

64. Punch biopsy
A piece of tissue may
be removed under
local anesthetic with
a disposable skin
punch for histology,
culture and the direct
demonstration of
amastigotes.

65. Parasitised macrophage in skin section
Many parasitised
macrophages can
be seen in this
section from an
acute lesion
caused by L.
major.

66. DIAGNOSIS
1.1.Needle aspirationNeedle aspiration
Bone – sternum – liver, spleenBone – sternum – liver, spleen
Histology – 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 cell Progressive fall with the red cell
countcount

67. •Formalin gel (aldehydeFormalin gel (aldehyde))
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. Other negative after cure. Sometimes + or – lung. Other
diseases.diseases.

68. 6.6.Fluorescent antibodyFluorescent antibody
•IV + V:IV + V: In trypanosomal infection In trypanosomal infection
•Skin test (Montenegro)Skin test (Montenegro)
Delayed hypersensitivity reactionDelayed hypersensitivity reaction
0.2 ml. suspension0.2 ml. suspension

69. TREATMENT
•Antimonial Antimonial – –
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, diarrhea Abdominal pain, diarrhea

70. ContraindicationContraindication:: Liver and kidney Liver and kidney
failurefailure
ii.ii. Sodium stibogluconate (Pentostam Sodium 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

71. Treatment
Sodium stibogluconateSodium stibogluconate
Pentavalent antimony (SbPentavalent antimony (Sb
+5+5
))

72. demonstration of amastigotes(L. donovani bodies) demonstration of amastigotes(L. donovani bodies)
intracellular form is usually 2-4 micron consists of intracellular form is usually 2-4 micron consists of
nucleus and is rod like with a homogeneous mass nucleus and is rod like with a homogeneous mass
of cytoplasm,while the extracelluar or culture of cytoplasm,while the extracelluar or culture
form is14-20 microns in length 1.5 to 3.5 breadthform is14-20 microns in length 1.5 to 3.5 breadth
L. donovani bodyL. donovani body

73. 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,secondary Secondary – in TB,secondary
carcinoma,hodgkins disease,leukaemia,and carcinoma,hodgkins disease,leukaemia,and
variety of other conditions.variety of other conditions.
Myeloid metaplasia ,spleenomegaly Myeloid metaplasia ,spleenomegaly
hepatomaegaly etc etchepatomaegaly etc etc

74. GAUCHER’S DISEASE

75. History
Discovered by Philippe Gaucher, a medical Discovered by Philippe Gaucher, a medical
student in Paris, in 1882.student in Paris, in 1882.
He was studying a woman with an enlarged He was studying a woman with an enlarged
spleenspleen

76. DefinitionDefinition
Gaucher's (go-SHAYZ) disease occurs when Gaucher's (go-SHAYZ) disease occurs when
certain harmful fatty substances build to certain harmful fatty substances build to
excessive levels in your liver, spleen, lungs, excessive levels in your liver, spleen, lungs,
bone marrow and, less commonly, your brain. bone marrow and, less commonly, your brain.
This accumulation of fatty material in tissues This accumulation of fatty material in tissues
interferes with the normal functioning of interferes with the normal functioning of
organs, and may cause organ enlargement organs, and may cause organ enlargement
and bone pain.and bone pain.

77. Gaucher's disease results from an enzyme Gaucher's disease results from an enzyme
deficiency, and sometimes the term deficiency, and sometimes the term
"glucocerebrosidase deficiency" is used to "glucocerebrosidase deficiency" is used to
describe this condition.describe this condition.
Gaucher's disease is most common in Eastern Gaucher's disease is most common in Eastern
and Central European (Ashkenazi) Jews. It and Central European (Ashkenazi) Jews. It
can occur at any age in life, and affects males can occur at any age in life, and affects males
and females approximately equally.and females approximately equally.

78. What Is It?
Gaucher’s DiseaseGaucher’s Disease- rare inherited metabolic - rare inherited metabolic
disease or disorder ; due deficiency or lack of an disease or disorder ; due deficiency or lack of an
enzyme called enzyme called GlucocereborsidaseGlucocereborsidase
Results in an accumulation of Results in an accumulation of glucocerebrosideglucocerebroside
within 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 disease Severity of the disease can vary and thus disease
divided into following typesdivided into following types

79. TYPE 1
Most common and can begin at any ageMost common and can begin at any age
1 in 10,000 1 in 10,000
Patients are bruised very easily Patients 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

80. 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

81. TYPE 2
Rarest of all the typesRarest of all the types
Appears during the first few months of life Appears during the first few months of life
in a babyin a baby
Great brain damage – mental retardationGreat brain damage – mental retardation

82. 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

83. TYPE 3
Begins in childhoodBegins in childhood
Liver and spleen enlargement Liver and spleen enlargement
Causes bone marrow and damages the Causes bone marrow and damages the
central nervous systemcentral nervous system
Mental retardation is quite commonMental retardation is quite common
Usually die around the ages 15-30 Usually die around the ages 15-30

84. Symptoms
Bone painBone pain
Victim bruises easily and there are many Victim bruises easily and there are many
fractures in their bonesfractures in their bones
Difficulty walkingDifficulty walking
Blood clottingBlood clotting

85. Symptoms (continued)
Muscle weaknessMuscle weakness
Poor coordinationPoor coordination
SeizuresSeizures

86. How Do You Get It?
Acquired if both parents of the disease are Acquired if both parents of the disease are
carrierscarriers
A victim receives an abnormal form of the A victim receives an abnormal form of the
genes- a genes- a Gaucher GeneGaucher Gene from both parents from both parents
CarrierCarrier- person with one normal gene and - person with one normal gene and
one Gaucher Gene ( a carrier will not show one Gaucher Gene ( a carrier will not show
signs of the disease)signs of the disease)

87. If 2 Carriers Have Children
There is a one in four chance of a There is a one in four chance of a
child inheriting the diseasechild inheriting the disease

88. Who Has It?
10,000 to 20,000 Americans10,000 to 20,000 Americans
High rate found in the Ahkenazi Jewish High 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 Types 2 & 3 are found in 1 out of
50,000-100,000 births50,000-100,000 births
Type 3-mainly found in people of northern Type 3-mainly found in people of northern
Swedish AncestrySwedish Ancestry

89. Prevention
No real preventionNo real prevention
Genetic Counseling is recommended for Genetic Counseling is recommended for
parents with a family history of the diseaseparents with a family history of the disease

90. Treatments
No No CURECURE
Enzyme replacement therapy-injections of Enzyme replacement therapy-injections of
the enzyme the 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

91. Hairy cell leukaemia
Hairy cell leukaemia is an uncommon Hairy cell leukaemia is an uncommon
disorder of middle and late adult life.disorder of middle and late adult life.
Characterized by the presence in bone Characterized by the presence in bone
marrow,spleen,and peripheral blood of marrow,spleen,and peripheral blood of
abnormal mononuclear cells with hairy abnormal mononuclear cells with hairy
cytoplasmic projections and best detected cytoplasmic projections and best detected
by phase contrast microscopy.by phase contrast microscopy.

92. Males affected more than femalesMales affected more than females
Marked by splenomegaly , however Marked by splenomegaly , however
lymphadenopathy is unusual lymphadenopathy is unusual
Splenectomy is usually regarded as Splenectomy is usually regarded as
treatment of choicetreatment of choice
Long term use of injections of alpha Long term use of injections of alpha
interferon cause regression of hairy cellsinterferon cause regression of hairy cells

93. Hairy cell leukemia is actually a mature B Hairy cell leukemia is actually a mature B
cell neoplasm. It is usually classified as a cell neoplasm. It is usually classified as a
sub-type of chronic lymphoid leukemia for sub-type of chronic lymphoid leukemia for
convenience. It is uncommon, representing convenience. It is uncommon, representing
about 2% of all leukemias, or less than a about 2% of all leukemias, or less than a
total of 2000 new cases diagnosed each year total of 2000 new cases diagnosed each year
in the North America and Western Europe in the North America and Western Europe
combined. combined.

94. Originally known as histiocytic leukemia, Originally known as histiocytic leukemia,
malignant reticulosis, or lymphoid malignant reticulosis, or lymphoid
myelofibrosis in publications dating back to myelofibrosis in publications dating back to
the 1920s, this disease was formally named the 1920s, this disease was formally named
leukemic reticuloendotheliosis leukemic reticuloendotheliosis

95. 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 the build up in the bone marrow, which means that the
bone marrow has difficulty producing enough bone marrow has difficulty producing enough
normal cells: white blood cells to fight infections, normal cells: white blood cells to fight infections,
red blood cells to carry oxygen, and platelets to red blood cells to carry oxygen, and platelets to
stop bleeding. Consequently, patients usually stop bleeding. Consequently, patients usually
present with infection, anemia-related fatigue, present with infection, anemia-related fatigue,
and/or easy bleeding.and/or easy bleeding.

96. Hematologic Disorders Causing
Massive Splenomegaly
 Polycythemia VeraPolycythemia Vera
 Multiple MyelomaMultiple Myeloma
 POEMS SyndromePOEMS Syndrome
 Waldenström's MacroglobulinemiaWaldenström's Macroglobulinemia
 chronic lymphocytic leukemiachronic lymphocytic leukemia
 non-Hodgkin lymphomanon-Hodgkin lymphoma
 chronic myelocytic leukemiachronic myelocytic leukemia
 malaria (hyper-reactive malarial malaria (hyper-reactive malarial
splenomegaly)splenomegaly)