Lymphatic disorders, lymphedema lymphomas and lymphangitis ppt

1. Lymphatic Disorders
Dr.Reetinder Kaur Chahal
Associate Professor
GMC and Rajindra Hospital,Patiala

2. Anatomy of lymphatic system
• Components of the lymphatic system include
1. initial /terminal lymphatic capillaries (absorb
lymph)
2. Collecting lymphatic vessels ( lymph
transport)
3. lymph nodes, lymphatic cells, and a variety of
lymphoid organs

5. Lymphatic circulation
• Lymph flows through lymph capillaries
(unfiltered lymph) large lymphatic vessels to
lymph nodes (filter lymph) to efferent
lymphatics to 2 major lymphatic channels :
right lymphatic duct and thoracic duct to
venous system

6. Composition of lymph
• Lymph is clear transparent yellow alkaline fluid
similar to blood plasma
• Transports absorbed fat of digestion in
intestine becomes whitish and is called chyle.

7. Functions
3 main functions :
• Extracellular fluid drainage :
– ULTRAFILTERATE at capillary level is reabsorbed and
transported back to circulation.
(Macromolecules and intravascular protiens)
• FIRST LINE OF IMMUNE SYSTEM:
– antigens microbes, mutant cells are presented to
lymph nodes
• Fat absorbtion and transport to circulation.

8. Microcirculation
• Slow rhythm, low velocity and low pressure
• Centripetal forward flow of lymph maintained
by
– Intrinsic contractility
– Valve mechanism
– Others: muscle activity, -ve pressure, transmitted
arterial pressure

10. LYMPHEDEMA

11. Definition
• Lymphedema is abnormal limb swelling
caused by accumulation of increased amount
of high protein ISF,
• Secondary to defective lymphatic drainage in
presence of normal capillary filteration

12. Pathophysiology and staging
• Lymphedema :
– Swelling of one or limbs / trunk/genitalia
– Caused by progressive accumulation of protein
rich interstitial fluid.
– Due to impairment of lymphatic vascular function

13. Physical Examination
• Inspection: asymmetry, peau d’orange
• Palpation: pitting, Stemmer’s sign
• Measure limb circumference and volume

14. Clinical Staging (ISL)
• Stage 0: Latent
• Stage I: Reversible swelling
• Stage II: Irreversible, non-pitting
• Stage III: Elephantiasis

15. Differential Diagnosis
• Deep vein thrombosis
• Chronic venous insufficiency
• Lipedema
• Myxedema
• Soft tissue tumors

16. Pharmacologic Treatment
• Antibiotics for cellulitis
• Antifilarials: DEC, ivermectin, albendazole
• Diuretics not routinely recommended

17. Primary lymphedema
• Cause is unknown
• Rare
• Classified based on age of onset .
1. Congenital lymphedema (Milroy’s ds)
– Age of onset < 2 years
2. Lymphedema Praecox (Meige’s ds)
– Age 2-35 years (puberty)
3. Lymphedema Tarda
– Onset beyond 35 years

18. Pathology – PRIMARY LYMPHEDEMA
1. Aplasia – lymphatics not developed at all /
absent . Seen In MIROY’S ds.
2. Hypoplasia – lymphatic channel underdeveloped.
– Vessels and nodes are few in no. and of small diameter
leading to inadequate drainage
– Mc type of abnormality.
3. Heperpalsia – excessive no. of lymphatics with
impaired function.
– Enlarged and distorted lymphatics

19. Secondary lymphedema
• Most common form
• Causes can be
1. Post traumatic/ tissue damage/surgery
– LN excision
– Radiotherapy
– Burns
– Varicose surgery
2. Malignant ds
– LN mets
– Infiltrative CA
– Lymphoma
3. Venous disorders
– Chronic insufficiency/ venous ulcers/ iv drugs
4. Infections
– FILARIASIS
– CELLULITIS
– TB/ LYMPHADENITIS
5. Inflammation

20. Clinical features
• EDEMA : excessive protein rich fluid in intersitium
– Pitting edema: initial stages
– Square toes: edema extends to distal feet
– Buffalo hump: dorsum of forefoot involved
– Indurated and fibrotic : late stages
• Skin changes :
– Lichenification / peau d’orange/ eczematous
• Other :
– Heaviness of limb
– Lymphangitis

22. Complications
1. Infections : lymphangitis
2. Malnutrition and immunodeficiency
– Due to Protien loss
3. Malignancy
– Lymphangiosarcoma
– Scc
– Malignant lymphoma
– Melanoma
• Lymphangiosarcoma –
– Rare
– Long standing lymphedema
– Eg, : Postmastectomy – STEWART TREVES Synd.
– Needs amputation.

23. Clinical staging
spontaneous spontaneously
(reversible) (irreversible)

24. • (A) Stage 0: subclinical lymphedema;
• (b) Stage 1: Pitting edema;
• (c) Stage 2: tissue fibrosis;
• (d) Stage 3: Skin changes.

26. Diagnostic tests
• Always exclude others causes of limb swelling.
1. Routine test:
– CBC RFT LFT 2D ECHO/
– BLOOD SMEARS FOR MICROFILARIAE
– USG/DOPPLER
2. SPECIFIC INV.
– LYMPHOSCINTIGRAPH (ISOTOPE)
– CONTRAST LYMPHANGIOGRAPHY
– MRI/ CT

27. LYMPHOSCINTIGRAPHY
• ISOTOPE BASED STUDY
• METHOD:
– Tc99m labelled sulfur colloid/ Radioiodinated human
albumin injected into 1st
interdigit space. Taken up by
lymphatics. Monitered by whole body gamma camera 30-
60 min. after injection
– Assesses lymphatic funct. : rate of clearance of radiotracer
i.e ability to transport large radiolabeled protein from
interstitial space to nodal basin to vascular compartment
– Uptake < 0.3% of total dose at 30 min. is diagnostic
– Normal uptake: 0.6%-1.6%

30. Contrast Lymphangiography
• Invasive study
• Cannulation of lymphatics at foot – contrast
injected – visualized under xray / mri/ ct
• Detailed lymphatic anatomy
• Contrast material used – lipidol/ ICG Fluorescent
dye/ Gadolinium based
• Icg lymphangiography uses infrared camera to
detect the fluorescence.
• Reserved for preoperative evaluation.

31. ICG lymphangiography

32. MANAGEMENT
1. NON- OPERATIVE
GOALS of treatment are:
– Reduce swelling
– Prevent complications
– Mainstay in management of lymphedema
– Surgery reserved for failed medical mx.

33. Edema preventive measures

34. • Standard of care for lymphedema is k/a: COMPLEX
DECONGESTIVE THERAPY
• 4 COMPONENTS:
1. SKIN CARE
2. MANUAL DECOMPRESSION DRAINAGE (MLD)
3. MULTILAYER LYMPHEDEMA BANDAGING
4. EXERCISES
• 2 PHASES:
– PHASE 1: INTENSIVE PHASE
 done by trained therapist
 mobilizing the lymphatic load and initiate reduction of fibrosis
– PHASE 2: MAINTAINANCE PHASE
 Optimizing and preserving the achieved state

35. Contraindication of CDT
• Acute cellulitis/ erysipelas
• Acute thrombophlebitis
• Decongested heart failure
• PAD

36. Skin care
• Includes
– Limb wash hygiene
– Drying
– Use moisturizer
• Antifungals, antibacterials to prevent cellulitis
• keratolytics

37. Manual Lymphatic drainage

38. Multilayered Lymphedema Bandaging
• Maintain tissue hydrostatic pressure
• 2 types of pressure are produced
1. Low resting pressure
2. High working pressure- during muscle
contraction
• Pressure must be graduated
– 100% ankle, 70%foot, 50% midthigh, 40% groin

40. Intermittent Pneumatic Compression
• Multichamber pumps remove excess fluid by
decreasing capillary filteration

41. Exercise
• Pumping action of muscle
• Intrathoracic negative pressure
• Weight reduction

42. Surgical managent
• 2 categories:
1. Reconstructive/ bypass procedure
 Lymphatico –lymphatic bypass
 Lymphatico –venous bypass (LVA)
 Lymphnode – venous bypass
 Vascularized LN Transfer (VLNT)
2. Excisional / reduction procedures
 SISTRUNK procedure
 CHARLES procedure
 HOMAN procedure
 THOMPSON procedure
 LIPOSUCTION
 SURGICAL DEBULKING

43. Patient selection
• Stage II/III lymphedema
• Recurrent lymphangitis
• Failed CDT/ Medical therapy
• Contraindication to CDT/
• Association with venous insufficiency
• SEVERE functional impairment
• BMI>30

44. LYMPHO-VENOUS ANASTOMOSIS
• Considered for
– Proximal obstruction with preserved , dilated
distal peripheral lymphatics
– The residual dilated lymphatics are anastomosed
with nearby veins/ harvested lymphatic channel
– Commonly done for extremity lymphedema
Post axillary lymphadenectomy/ inguinal or pelvic
lymphadenectomy

46. Prior localization of
lymphatics can be done with
ICG mapping and lymphatics
visualized intraoperatively by
distal injection of isosulphan
blue dye.
LYMPHATICOVENOUS BYPASS

47. VLNT
(a) Vascularized
lymph node
transfer from
supraclavicular
region;
(b) vascularized
lymph node
transfer to the
ankle.
Transfer of functional lymph nodes with their blood vessels to a
diseased area where native nodes have been removed and
restoring their blood supply through microvascular anastomosis
to recipient site blood vessels

48. HOW DOES VLNT WORKS ?
• LYMPHANGIOGENESIS- promots growth and
connection of new lymphatics
• Transplanted LN acts as lymph pump

49. REDUCTIVE PROCEDURES
SURGICAL DEBULKING OF LYMPHEDEMA

53. CHARLES PROCEDURE

54. LIPOSUCTION

55. Lymphatic Filariasis
• Vector borne caused by-
– Wuchereria bancrofti 90%
– Brugia malayi 10%
• Mosquito
– Culex ( MC vector)
– Anopheles
– Aedes

57. Diagnosis
• Night blood examination to detect microfilarie
• Immuno- chromatographic card test
• Ultrasound for locating adult worms
– (negative once lymphedema establishes)

58. Management
• DEC- Diethylcarbamazine
– Microfilaricidal + adult worms
– Dose – 6mg/kg wt x 12 day regime
Or single dose regime
– Only effective in acute phase of disease when
lymphedema not established
• Combination:
– DEC+ ALBENDAZOL
– DEC + ALBENDAZOL+ IVERMECTIN

59. Surgical Management
• Indications: failed conservative treatment
• Excisional: Charles, Homans procedures
• Physiologic: Lymphovenous bypass, VLNT
• Liposuction: for solid-phase lymphedema
• Post-op care is critical

60. Prognosis & Follow-Up
• Chronic and progressive condition
• Requires lifelong management
• Patient education is essential

61. Summary
• Early diagnosis improves outcomes
• Conservative management is first-line
• Surgery reserved for advanced or refractory
cases
• Multidisciplinary approach is key

62. LYMPHANGITIS

64. When pathogenic organisms enter the lymphatic channels
local inflammation and subsequent infection ensue,
manifesting as red streaks on the skin.
The inflammation or infection then extends proximally
toward regional lymph nodes.
Bacteria can grow rapidly in the lymphatic system

65. Definition
Inflammation of the lymphatic channels that
occurs as a result of infection at a site distal to
the channel

66. Etiology
• Species of group A beta-hemolytic streptococci (GABHS) (MC)
• Staphylococcus aureus
• Pseudomonas species
• Streptococcus pneumoniae
• Pasteurella multocida
• Gram-negative rods, gram-negative bacilli, and fungi
• Aeromonas hydrophila
• Wuchereria bancrofti
Diabetes, immunodeficiency, varicella, chronic steroid use, or other
systemic illnesses have increased risk of developing serious or rapidly
spreading lymphangitis.

67. Nodular lymphangitis
Superficial inoculation with one of the following organisms:
– Sporothrix schenckii
– Nocardia brasiliensis
– Mycobacterium marinum
– Leishmania panamensis
– L guyanensis
– Francisella tularensis

68. Prognosis
• With uncomplicated lymphangitis is good.
• Antimicrobial regimens are effective in more than 90% of
cases.
• Without appropriate antimicrobial therapy cellulitis may
extend along the channels; necrosis and ulceration may occur.
• Morbidity and mortality is related to the underlying infection.
• Mortality associated with lymphangitis alone, lymphangitis
caused by GABHS can lead to bacteremia, sepsis, and death.

69. Clinical presentation
• H/o minor trauma to an area of skin distal to the site of
infection
• Children with lymphangitis: fever, chills, and malaise,
headache, loss of appetite, muscle aches.
• H/o recent cut or abrasion or of an area of skin
• Can progress rapidly to bacteremia and disseminated
infection and sepsis

71. Physical examination
• Erythematous and irregular linear streaks extend from the primary
infection site toward draining regional nodes.
• Primary site may be an abscess, an infected wound or an area of
cellulitis
• Blistering of the affected skin may occur
• Lymph nodes associated with the infected lymphatic channels are
often swollen and tender
• Patients may be febrile and tachycardic

72. Differential diagnosis
• Contact dermatitis
• Cellulitis
• Septic thrombophlebitis
• Superficial thrombophlebitis
• Necrotizing fasciitis
• Myositis
• Sporotrichosis

73. Investigations
• Complete blood cell (CBC) count
• Blood culture
• Leading -edge culture or aspiration of pus
• Cultures and Gram staining of fluid.
Threshold sensitivity of Gram staining: 100,000 microorganisms per
milliliter, concentration rarely found in cellulitis or lymphangitis.

74. A study found that multidetector computed tomography (MDCT)
imaging was very useful in determining the morphology
(cellulitis with a few small subcutaneous nodules and channels)
and the extension of the lesion in a case of nodular lymphangitis
caused by Mycobacterium marinum

75. Management
• Antibiotics
• Oral
• Parenteral (with signs of systemic illness (eg, fever,
chills and myalgia, lymphangitis).
• Analgesics
• Anti -inflammatory
• Elevation and immobilization of affected areas reduces
swelling, pain, and the spread of infection. An abscess
may require surgical drainage.

76. Nodular lymphangitis
• Treatment of nodular lymphangitis is determined by
identifying the underlying cause.
• Sporotrichosis is most often identified in this disease
and is commonly found among gardeners.

77. Antibiotics
• Dicloxacillin
• Cephalexin
• Cefazolin
• Cefuroxime
• Ceftriaxone
• Clindamycin
• Nafcillin
• Trimethoprim and sulfamethoxazole (TMP/SMZ)

78. LYMPHOMAS

79. They are progressive neoplastic condition of
lymphoreticular
system arising from stem cells.
• Lymphomas are the 3rd most common
malignancy among children comprising 15% of
paediatric cancers.

80. Aetiology
• Genetic predisposition.
• Sjogren’s syndrome – 30 fold increase of NHL.
• HIV infection.
• Wiskott – Aldrich syndrome.
• Ataxia – Telangiectasia.
• Bloom’s syndrome.
• Virus etiology – Epstein Barr virus.
• Celiac sprue – intestinal T cell lymphoma.
• H pylori may be associated with MALT lymphoma.
• Occupation causes – hair dye workers; herbicide
exposure.
• Ionizing radiation.
• Smoking; alcohol consumption; tobacco usage.
• Lymphomas are more common in western countries than in asia

81. Types
• • Hodgkin’s lymphoma (HL).
• • Non-Hodgkin’s lymphoma (NHL

82. WHO modified REAL classification (Revised
European
American Lymphoma) of lymphoma:
1. B-cell neoplasms
I. Precursor B cell neoplasm—ALL, LBL.
II. Peripheral B cell neoplasm—It includes all B
cell
related non-Hodgkin’s lymphomas
2. T-cell and putative NK cell neoplasms
I. Precursor T cell neoplasms—ALL and LBL
T cell related.
II. Peripheral T cell and NK cell neoplasm—It
includes all T cell related non-Hodgkin’s
lymphomas.
3. Hodgkin’s lymphoma
I. Predominant HL—Nodular lymphocyte type
II. Classical HL
Nodular sclerosis
Lymphocyte rich
Mixed cellularity
Lymphocyte depletion
Note: ALL is acute lymphoblastic leukaemia.
LBL is
lymphoblastic lymphoma.

83. Clinical Features
• It is more common in males.
• It has got bimodal presentation. It is seen in
young and adolescents (20-30 years) as well as
in elderly(> 50 years).
• Painless progressive enlargement of lymph
nodes.They are smooth, firm, nontender,
typically Indiarubber consistency.

84. Site
• Cervical lymph nodes commonest—82%
(lower deepcervical group and in posterior
triangle).
• Others include axillary, mediastinal,
inguinal,abdominal.

85. Specific Features
• Nodular sclerosis is most commonest type.
• Consecutive group of lymph nodes are
involved.
• Splenomegaly is very common (45%).
• Hepatomegaly with jaundice—jaundice is due
tohaemolysis or due to diffuse liver
involvement

86. • Constitutional symptoms like fever, pruritus, weight loss
may be present which signifies stage “B”, which has got
poor prognosis. Stage “A” is absence of these symptoms
which signifies better prognosis.
Stage ‘B’ symptoms:
• Weight loss, more than 10% in 6 months.
• Fever, earlier called as Pel Ebstein fever is actually
is due to brucellosis.
• Pruritus
• Anaemia
• Bone pain
• Mediastinal lymph node involvement may cause
compression features like SVC obstruction.
• Occasionally bone like vertebrae may get involved.
• Anaemia, pancytopaenia.

87. Ann-Arbor Clinical Staging:
Stage 1: Confined to one group of
lymph node.
Stage 2: More than one group of
lymph nodes on
one side of the diaphragm.
Stage 3: Nodes involved on both
sides of the
diaphragm.
Stage 4: Extra nodal involvement
like liver, bone
marrow.
Suffix ‘S’—Spleen involved
Suffix ‘B’—Presence of
constitutional symptoms
Suffix ‘A’—Absence of
constitutional symptoms

88. Differential Diagnosis
• Tuberculosis.
• NHL.
• HIV.
• Chronic lymphatic leukaemia.
• Nonspecific lymphadenitis.
• Sarcoidosis.
• Secondaries in lymph nodes.

89. Investigations
• Blood: Hb%, ESR, peripheral smear, blood
urea,
serum creatinine.
• FNAC of lymph nodes.
• Excision biopsy of lymph nodes. Full lymph
node isexcised to retain the architecture of
the lymph node.
• It is important to grade the tumour.

90. • Chest X-ray—to look for mediastinal lymph nodes,
pleural effusion.
• U/S abdomen—to look for the involvement of liver,
spleen, abdominal lymph nodes.
• CT scan of mediastinum and abdomen better method.
• Lower limb lymphangiography to look for the pelvic
and retroperitoneal lymph nodes.
• Bone marrow biopsy to stage and also to see
theresponse to treatment.

91. • Staging laparotomy: The abdomen is opened.
Splenectomy is done mainly to remove the tumour
bulk, as spleen is commonly involved and also to
avoid irradiation to splenic area which often causes
unpleasant pulmonary fibrosis. Biopsies are taken
from both lobes of the liver (needle biopsy) from
paraaortic, celiac mesenteric, iliac nodes. In females
ovaries are fixed behind the uterus to prevent
radiation oophoritis (oopheropexy/ovarian translocation).
Staging laparotomy is not routinely done now. It
is done only if it benefits the patient to have better
plan of treatment or better result

92. Treatment for HL
• Treatment strategy for HL
• Early favourable stage – extended field
radiation only
• Early unfavourable stage – extended radiation
plus chemotherapy
• Advanced stage – extensive chemotherapy
often with local radiation

93. Unfavourable signs in early HL
• • Large mediastinal mass
• • Extranodal disease
• • Elevated ESR
• • Four or more involved regions
• • Presence of B symptoms
• • Anaemia; low serum albumin level
• • Age more than 50
• • Male gender
• Note: First five parameters are most important

94. • • Stage I and II:
• 1. Mainly radiotherapy—External high cobalt RT.
• • Above the diaphragm—“Y” field therapy,
• covering cervical, axillary, mediastinal lymph
• nodes.
• • Below the diaphragm, mantle or inverted “Y”
• field therapy, covering paraaortic and iliac
• nodes.
• 2. Chemotherapy is also given

95. • • Stage III and IV: Mainly chemotherapy.
• Drugs used include—
• • Mustine. M. (Mechloroethamine).
• • Oncovine. O. (Vinca alkaloids).
• • Procarbazine. P.
• • Prednisolone. P.
• Other regimens available — MVPP, ABVD

96. NON-HODGKIN’S LYMPHOMA (NHL)
• It occurs in middle aged and elderly. It is more
aggressive than HL.
• It involves asymmetrical group of lymph nodes.
• General condition is poor.
• Inner Waldeyer ring, epitrochlear lymph nodes,
peripheral lymph nodes are commonly involved.
• Spleen is not commonly involved.
• Hepatomegaly is common.
• Vertebral involvement is common; paraplegia can
occur.
• Secondary infection, cachexia and immunosuppression
is more common.

97. • Types
• • Nodular (Follicular)
• • Diffuse lymphocytic
• • Undifferentiated
• • Histiocytic type.

98. • Treatment
• Mainly chemotherapy
• Various regimens available include:
• • ChOPP—Chlorambucil, Oncovin, Procarbazine,
• Prednisolone.
• • ABVD—Adriamycin, Bleomycin, Vincristine,
• Dacarbazine.
• • ABVP—Adriamycin, Bleomycin, Vincristine,
• Prednisolone.
• • Combinations of above.
• Role of radiotherapy in NHL: When vertebra is involved.
• Prognosis is poor compared to HL.

99. BURKITT’S LYMPHOMA
(Malignant Lymphoma of Africa)
• • It is common in South Africa and New Guinea.
• • Epstien Barr virus may be the etiological agent. It is
• common in children.
• • It is associated with infectious mononucleosis.
• • It is common in malaria endemic area.
• • The tumour is multifocal, rapidly growing, painless.
• • Different groups of lymph nodes can also be affected

100. Microscopy
• Primitive lymphoid cells with large clear histiocytes—
• starry night (starry sky) pattern.
Sites
• • It is common in jaw—either lower or upper.
• • Abdominal presentation and renal involvement is
• common (75%).
• • Renal involvement often may be bilateral.
• • In females, ovaries are commonly affected.

101. • Types
• 1. Endemic (African)—Commonly occurs in
jaw
• 2. Nonendemic (sporadic)—Commonly occurs
in
• abdomen
• 3. Aggressive lymphoma—Occurring in HIV
patients.

102. • Investigation
• • FNAC and biopsy confirms the diagnosis.
• • Jaw X-ray shows osteolytic lesions.
• • U/S abdomen is done to look for
involvement of
• kidneys.
• • Blood urea and serum creatinine estimation
is done.

103. Treatment
• • Radiotherapy.
• • Chemotherapy: Cyclophosphamide,
Methotrexate,
• Orthomelphalan.
• • Surgery is usually not indicated unless it is
localised
• or in case of involvement of ovaries.
• Prognosis is good.

104. CUTANEOUS T CELL LYMPHOMA
• Cutaneous T cell lymphoma comprises mycosis
fungoides, Sezzary syndrome, reticulum cell sarcoma
of skin and other cutaneous lymphocytic dysplasias.
Mycosis fungoides is the commonest among them.
• Cutaneous T cell lymphoma can be indolent
(commonly mycosis fungoides); aggressive (Sezzary
syndrome); provisional (granulomatous/panninculitis
like T cell lymphoma).
.

105. • Initial macular patch/plaque phase slowly
changes into tumour phase with painful,
pruritic erythroderma often with visceral
spread. Alopecia mucinosa and follicular
mucinosis are common in mycosis fungoides.
• Lymph nodes may get involved.
• Tumorcells in peripheral smear are also
important in deciding therapy and prognosis

106. • Multiple skin biopsies/peripheral smear/node
biopsy/immunohistochemistry/pheo or genotyping
are important investigations
• Prognosis depends on extent of skin involvement
(more the 10% body surface area carries poor
prognosis)/nodal spread/blood spread.
• Treatment: Localised external beam radiotherapy;
topical chemotherapy (bexarotene gel/carmustine
ointment); phototherapy; total skin electron beam
therapy; extracorporeal photochemotherapy. Bexarotene
is a type of retinoid.

107. Sezzary syndrome
• is a type of cutaneous T cell
• lymphoma with skin lesions with special Sezzary
• cells having cribriform nucleus. It is often
associated
• with leukaemias. It is treated like any other
cutaneous
• T cell lymphoma.