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214
Manipal Manual of Surgery
• It is due to an inherited abnormality of the lymphatic
system.
• A few cases which occur later in life may be due to
unnoticed factors—repeated bacterial and fungal
infections, trauma to feet, etc.
• Loss of venoarteriolar reflux (VAR), which protects
the lower limb capillaries from excessive hydrostatic
forces in the erect posture, occurs in advanced age.
Diseases such as diabetes and chronic venous
insufficiency may also contribute.
Causes of Primary Lymphoedema
Hereditary: Associated with syndromes such as
I.
Turner’s (XO), Klinefelter’s (XXY), Down’s
(Trisomy 21).
II. Familial
1. Nonne-Milroy—type I (congenital)
• Occurs in 1 of 6000 live births
• Inherited as an autosomal dominant trait
• Brawny lymphoedema of both legs, genitalia,
arms, face, etc. develops from birth (Fig. 29.4).
• Onset: <2 years of age
. Meige’s disease—type II (praecox)
2
• Lymphoedema develops between puberty
and middle age (50 years). It may also involve
the arms.
• A few are inherited in an autosomal dominant
manner.
• Onset: 2–35 years of age.
. Tarda onset after 35 years of age
3
Pathophysiology (Fig. 29.5)
Lymphoedema leads to impaired immune surveillance and
predisposes to other malignancies (Key Box 29.2).
Types of Primary Lymphoedema
• Lymphatic aplasia: Number of lymphatic channels
and nodes are grossly reduced.
• Lymphatic hypoplasia: Lymphatic channels are of a
small calibre.
• Milroy’s disease: A familial type of lymphoedema
congenita.
• Based on the time at which lymphoedema appears,
it is classified as either:
Birth: Lymphoedema congenita.
Puberty: Lymphoedema praecox.
Later: Lymphoedema tarda.
Fig. 29.4: Congenital lymphoedema—observe the right leg. One
attempt has been made to decrease lymphoedema by excision
Section II • General Surgery
Fig. 29.5: Pathophysiology of lymphoedema
Key Box 29.2
Malignancies Associated with Lymphoedema
Skin cancers
Squamous cell carcinoma
Malignant melanoma
Basal cell carcinoma
Sarcomas
Lymphangiosarcoma (Stewart-Treves syndrome)
Kaposi’s sarcoma
Liposarcoma
Malignant fibrous histiocytoma
Systemic disease
Lymphoma
Lymphatics, Lymph Vessels and Lymphoma
215
SECONDARY LYMPHOEDEMA (ACQUIRED)
1. Filarial elephantiasis (Fig. 29.6) is caused by
Wuchereria bancrofti, transmitted by the mosquito
Culex fatigans. The disease is caused by adult worms
which have an affinity towards lymphatic vessels and
nodes. Microfilariae do not produce any lesions.
• Initially, it causes lymphangitis which clinically
presents with high grade fever, chills and rigors,
red streaks in the limb, tenderness, and swelling
of the spermatic cord and scrotum (Table 29.1).
• The lymph nodes are swollen and tender. Retro-
peritoneal lymphangitis produces acute abdominal
pain.
• Due to repeated infections, fibrosis occurs and
results in lymphatic obstruction. Later, this gives
rise to lymphatic dilatation. Lower limb lymphatics
are dilated and tortuous (lymphangiectasis).
• Initially, lymphoedema is pitting in nature but
progressively becomes nonpitting.
Lymph (protein)
provides good nourishment for fibroblasts.
• After repeated infections, the skin over the limb
becomes dry and thickened, and is thrown into folds
and even nodules that break open and result in ulcers.
Hence, it is called elephant leg. Malnutrition and
infection precipitate lymphoedema. Oedema is also
due to reflux of lymph from para-aortic vessels into
the smaller lymphatics draining the lower limb. The
subcutaneous tissue is grossly thickened. The
presence of deep fascia prevents involvement of
the deep muscles of the lower limb (Key Box 29.3).
Key Box 29.3
Elephant Leg
2. After inguinal block dissection for secondaries in the
lymph nodes (upper limb lymphoedema following
axillary block dissection).
3. Following radiotherapy to lymph nodes.
4. Advanced malignancies.
5. Repeated infections due to barefoot walking (Fig. 29.7).
Symptoms (Table 29.2)
• Swollen limb, dull-aching pain
Fig. 29.6: Filarial lymphoedema
Table 29.1 Clinical manifestations of lymphatic filariasis
Site of involvement Acute Chronic
Lower limbs Lymphangiitis, lymphadeniitis Lymphoedema, chronic lymphadenitis
Scrotum Lymphangiitis Lymphoedema, chylocele
Spermatic cord Acute funiculitis Chronic thickened cord
Epididymis and testis Acute epididymo-orchitis Chronic epididymo-orchitis
Abdomen Acute retroperitoneal lymphangiitis Chyluria, lymphadenovarix
Breast Lymphangiitis Lymphoedema
• Tiredness, pins and needles, cramping pain
Section II • General Surgery
216
Fig. 29.7: Severe lymphangiitis
Manipal Manual of Surgery
• Loss of ankle contour
• Buffalo hump on the dorsum of the foot
• Toes are square
• Skin on the dorsum of the toes cannot be pinched
because of subcutaneous fibrosis—Stemmer’s sign
• In early cases, it pits on pressure. Later, oedema does
not pit and does not reduce even with elevation.
• Advanced cases: Chronic eczema, fungal infections
of skin (dermatophytosis) and nails (onychomycosis),
fissuring, warts.
• Ulceration is unusual.
• However, once ulcers start, recurrent bacterial infec-
tions are common, thus worsening the disease process.
Diagnosed by detecting: Microfilariae on blood smear
complement fixation test.
Treatment: Diethylcarbamazine (DEC) 100 mg bid ×
14 days.
Grades of Filarial Lymphoedema
Grade I Oedema pitting: Completely relieved on rest and
elevation. No skin changes.
Grade II Oedema pitting: Partially relieved on rest and
elevation. No skin changes.
Grade III Oedema nonpitting: Skin involvement, subcuta-
neous thickening present.
Grade IV Oedema nonpitting: Not relieved, warty projections,
elephantiasis, lymphorrhoea present.
Differential Diagnosis of Unilateral Elephantiasis of
the Leg
1. Filariasis is the most common cause of secondary
elephantiasis of the leg in endemic areas—coastal
Karnataka, coastal Andhra Pradesh, Tamil Nadu, etc.
(Table 29.3).
Table 29.2 Clinical features of lymphoedema
Early signs Change into Late/advanced cases—signs
1. Loss of ankle contour 1. Fibrosis 1. Chronic eczema
2. Buffalo hump on the dorsum of feet 2. Dermal thickening 2. Fungal infection—dermatophytosis
3. Stemmer’s sign: Skin on dorsum 3. Hyperkeratosis 3. Fungal infection of nails—
of 2nd toe cannot be pinched because onychomycosis
of subcutaneous fibrosis.
4. Early pitting on pressure, less on rest, elevation 4. Fissuring, verrucae, papilla
5. Squaring of toes 5. Grossly thickened leg with skin
Table 29.3 Comparison of primary and secondary lymphoedema
Primary lymphoedema Secondary lymphoedema
It is due to congenital aplasia and hypoplasia Filariasis is the common cause
Slowly progressive Rapidly progressive
It is seen in younger age group Middle age group
Females are more often affected Males are more commonly affected
Unilateral, begins distally, spreads proximally Sometimes, it can start proximally—unilateral or bilateral
Capillary haemangioma may be present Absent
Regional lymph nodes are absent Lymph nodes are grossly enlarged
Excisional operations are indicated Excisional operations and other types of surgery
Section II • General Surgery
Lymphatics, Lymph Vessels and Lymphoma
Table 29.4 Causes of secondary lymphoedema (Figs 29.8 to 29.10)
Cause Pathology
1. Infections Filariasis Recurrent lymphangiitis
Tuberculosis Lymphadenitis
Chronic lymphadenitis Lymphatic destruction
2. Surgery Lymph node block dissections (inguinal or axillary) Excis ion o f lymph nodes as part of
treatment
3. Lymph node obstruction Postradiotherapy large nodes—metastasis lymphoma Scarred and obliterated lymphatics
4. Tissue damage Burns scarring Loss of lymphatics
5. Venous diseases Chronic venous insufficiency, venous ulcer Lymphatic destruction
6. Endocrine Pretibial myxoedema Obliteration of initial lymphatics by mucin
217
2. Congenital AV fistula may present with unilateral
gigantism of the leg. Dilated veins, continuous
murmur, gigantism, and a nonhealing ulcer in the
leg in a young boy give the clue to the diagnosis.
3. Elephantiasis neuromatosa of the leg may cause
diffuse enlargement of the leg. The leg is tender on
palpation with a soft to firm diffuse swelling.
4. Extensive lipomatosis of the leg.
The causes of lymphoedema are summarized in
Key Box 29.4 and Table 29.4.
Investigations (
Lymphangiography)
By and large, no investigations are done in secondary
lymphoedema. In selected cases of primary lymphoedema, investigations may be done but are largely of
academic interest.
Key Box 29.4
Summary of Causes of Lymphoedema
Aplasia, hypoplasia (familial and genetic)
Parasitic (filariasis)
Lymph node obstruction—advanced malignancies
Altered motility—dysmotility (genetic)
Surgical extirpation—block dissection
Inflammatory/infection—recurrent
After radiotherapy, after barefoot walking ‘silica
particles’
Remember as APLASIA
LYMPHANGIOGRAPHY
A dye is injected into the lymphatics and the entire
draining lymphatics and lymph nodes are visualised.
Indications for Lymphangiography
1. Lymphoedema, if surgery is planned.
. Lymphoma, to detect pelvic nodes, para-aortic nodes,
2
etc. (but now CT is preferable).
Procedure
• Commonly, pedal lymphangiograms are done.
• 5–10 ml of methylene blue (patent blue) is injected
into the web spaces intradermally between the toes.
This delineates the lymphatics of the dorsum of the
foot which are identified. Then, an oily dye such as
“ultra fluid lipiodol” is injected (10–15 ml).
• It may take 12–24 hours to delineate the inguinal and
para-aortic nodes.
• Isotope lymphangiography refers to injection of
albumin labelled with technetium 99m colloid or
131
Results
• Metastases appear as irregular filling defects in the
lymph nodes.
• It may demonstrate hypoplasia or hyperplasia, as in
primary lymphoedema.
• If there is obstruction, the dye may return back
(dermal back flow).
Lymphangiographic Classification
Three types are recognised
1. Congenital hyperplasia (10%): This is a condition
wherein lymphatics are increased in number. These
megalymphatics are defective, resulting in chylous
ascites, chylothorax, and protein-losing enteropathy.
Being congenital, it manifests at birth. It is progressive,
involves the whole leg, and shows a variable response
to compressive therapy.
2. Distal obliteration (80%) (Key Box 29.5).
3. Proximal obliteration (10%)
• It is less common.
• Obstruction is at the region of the aortoiliac or
inguinal nodes.
• Whole leg/thigh is involved (Fig. 29.11).
• No family history.
• Rapid progression and poor response.
I.
Section II • General Surgery
218
Manipal Manual of Surgery
Fig. 29.8: A case of carcinoma of the
breast presented with lymphangiitis
Key Box 29.5
Fig. 29.9: Post-mastectomy lymph-
oedema of the right upper limb
Distal Obliteration (80%)
Distal lymphatics are commonly affected
In and around puberty—praecox involved
Sex: Common in females
Treatment is by compression, good response
Ankle, calf region is involved
Lymphatics are decreased or absent (aplasia)
Remember as DISTAL
Fig. 29.11: Unilateral limb oedema due to proximal obliteration
Complications of Lymphangiography
1. Lymphangiitis and toxaemia
• It is not routinely done nowadays because of the
availability of ultrasound, CT, and other noninvasive investigations.
2
. Rapid infusion should be avoided to prevent the risk
of lipid pulmonary embolus.
Lymphoscintigraphy
• It has a sensitivity and specificity of 92% and 100%,
Section II • General Surgery
respectively.
Fig. 29.10: Post-mastectomy lymphoedema—
progressive since 6 years (Courtesy: Dr Srijayan,
Professor, Department of Surgery, Calicut
Medical College, Calicut)
• It has replaced lymphangiography.
• Radiolabelled (technetium 99m) colloid is injected
into the web space between the 2nd and 3rd toes or
fingers. The limb is exercised periodically and images
are taken.
• If there is an abnormal accumulation of tracer with
collaterals, it is a sign of lymphoedema.
• MRI and CT are the latest investigations in addition
to lymphangiography for the evaluation of gross
swelling of the limb.
Treatment of Lymphoedema (Key Box 29.6)
Surgery has a small role in lymphoedema
Key Box 29.6
MLLB
Graduated pressure highest at ankle (100%) to lowest
in the groin (40%) should be applied.
It requires about 40–60 mmHg to leg and 30–40 mmHg
to arm.
MLLB should be worn at the start of the day and
removed at bed time.
However, limb should be kept elevated at night.
The aim is to get a graduated pressure in applying. If it
is applied wrongly, it may result in increase in lymphoedema.
I. CONSERVATIVE
1. Control of swelling: Decongestive lymphoedema
therapy (DLT)
• Bedrest, elevation
• Bandaging: Multilayered lymphoedema bandaging
(MLLB—Key Box 29.7).
• Compression garments
• Massage: Manual lymphatic drainage (MLD)
Lymphatics, Lymph Vessels and Lymphoma
219
Key Box 29.7
Guidelines for Treatment of
a Patient with Lymphoedema
L
Y
Manual lymphatic drainage (MLD) or massage
Prophylactic antifungal treatment to prevent athlete’s
Hygiene of skin and foot
Advice on diet—weight reduction
Treatment of infection—early and aggressive
Instructions about exercises
Compression garments
Surgery consultation as and when required.
Lymphoedema bandage is multilayered
}
foot
Remember as LYMPHATICS
• It aims at draining fluid and protein from ISF
space.
• It also stimulates lymphangion contraction.
2. Care of skin
• Wash daily with warm water
• Moisturiser or liquid paraffin should be applied
for dry skin
• Avoid injuries
• Hyperkeratosis should be treated by a keratolytic
agent such as 5% salicylic acid.
• Avoid ointments/herbal medicines/soaps, etc.,
which are allergic.
• Antifungal treatment
• Treat infections early and effectively.
3. Relief of pain
• Pain is multifactorial. It may be due to swelling,
infection, involvement of nerve/bone, etc.
• However, it is often psychosomatic and affected
by mood and morale.
• Antianxiety drugs may help.
4. Control of infections
• Usually it is due to streptococci/staphylococci.
• Oral or injectable penicillin and flucloxacillin or
clindamycin are the drugs of choice.
• Other drugs are co-amoxiclavulinic acid, cephalo-
sporins.
5. Exercises
• 40% of lymph is formed within skeletal muscle.
• It is directly proportional to central inflow.
• Slow systemic, isotonic movements such as
swimming and massage increase venous and
lymphatic return.
• Foot-end elevation of the bed.
6. Drugs
• Oxerutins are the drugs used for venous disease.
These are not yet licensed in the UK but are used
in India.
• They reduce capillary permeability, improve
microcirculatory perfusion, stimulate interstitial
macrophage proteolysis, and reduce erythrocyte
and platelet aggregation.
• Diuretics: 20 mg of furosemide every day/alternate
days. This helps in early cases of lymphoedema.
• Antifilarial treatment: Diethylcarbamazine citrate
(DEC) 100 mg 3 times/day for 21 days with every
attack of lymphangiitis and once in 6 months.
• Warfarin has been used to reduce lymphoedema
due to filariasis. It acts by enhancing macrophage
activity and extralymphatic absorption of interstitial fluid.
• Antibiotics are used in cases of cellulitis and
lymphangiitis.
II. SURGERY
Aim: To reduce the limb size.
1. Swiss-roll operation (Thompson’s): A skin flap
containing dermis is raised and buried into the deep
tissues (close to the vascular bundle). This is a dermal
flap prepared by denuding epidermis (Fig. 29.12).
2. Charles excision operation: It is indicated in primary
lymphoedema. It is performed for extensive swellings
and skin changes (Fig. 29.13). Thickened and diseased
skin and subcutaneous tissue are excised till the
healthy underlying structures are seen, followed by
split skin grafting. The skin has dermal lymphatics
which are never involved in filariasis. Thus, the subcutaneous lymph may flow via dermal lymphatics.
3. Nodovenous shunt: Dilated, enlarged inguinal
lymph node is anastomosed to a vein nearby, e.g. long
saphenous vein or femoral vein (Fig. 29.14).
Thus, these are 3 types of surgery commonly done
for filarial leg (Fig. 29.15). There are many other surgeries
which are of historical interest. However, the results of
surgery for filarial leg are disappointing. Many patients
develop intractable ulcers and wetting of the limb due
to loss of protein. The wound gets secondarily infected
resulting in sepsis, recurrent lymphangiitis, etc. As a
last resort many patients beg for amputation to get rid
of the ‘useless limb.’ After amputation, the limb can be
fitted with a prosthesis.
Lymphoedema and Chronic Venous Insufficiency (CVI)
• Superficial venous thrombophlebitis (SVT) and deep
vein thrombosis (DVT) can lead to lymphatic destruction and secondary lymphoedema. Lymphoedema
can also predispose to DVT.
Section II • General Surgery
220
Manipal Manual of Surgery
Fig. 29.12: Swiss-roll operation—
skin flap is raised
Fig. 29.13: Excision and grafting Figs 29.14A to C: (A) Node to vein, (B) Lympha-
tics threaded into a vein and (C) Microvascular
anastomosis
Fig. 29.15: Various surgeries for lymphoedema
• Some degree of superficial reflux may be present in
lymphoedema patients that must be managed
conservatively rather than by ‘blunder surgery.’
HODGKIN’S LYMPHOMA (HL)
Definition
It is a malignant neoplasm of the lymphoreticular
system. Thus, it can involve the lymph nodes, spleen,
and liver.
Risk Factors for Hodgkin’s Lymphoma
• History of infectious mononucleosis increases the risk
of HL two to threefold. Epstein-Barr virus (EBV) may
be a causative agent.
• Immunosuppression caused by the drugs increases
the risk of HL. The incidence of HL is significantly
higher in the HIV-infected population than in the
general population. Patients with systemic lupus
Section II • General Surgery
erythematosus and rheumatoid arthritis on immune
suppression and immunosuppression due to organ
transplantation have increased risk of Hodgkin’s
lymphoma.
• Genetic/molecular: At least 95% of HL represent
monoclonal B cell disorders.
Pathology
• The disease usually starts in one of the lymph nodes
as a painless swelling.
• Commonly, it involves the left supraclavicular region.
The nodes are enlarged and not matted. It spreads to
other nodes in a downstream lymphatic drainage
(contiguous spread).
• Cut surface of lymph nodes are smooth and homo-
geneous.
• The axial lymphatic system is almost always affected.
• Microscopy: ‘Cellular pleomorphism’—lymphocytes,
histiocytes, eosinophils, and fibrous tissue with Reed-
Sternberg cells, giant cells containing mirror-image
nuclei.
Lymphatics, Lymph Vessels and Lymphoma
WHO Classification/REAL (1994)—Revised European
American Lymphoma
I. Lymphocyte predominance, nodular (both
Hodgkin’s lymphoma and low grade B cell
lymphomas)
II. Classic Hodgkin’s lymphoma (HL)
• Lymphocyte-rich
• Nodular sclerosis—most common
• Lymphocyte depletion
• Mixed cellularity
However, classical HL and nodular lymphocyte
predominant HL—LPHL are now the more
practically used classification (Table 29.5).
Clinical Features
• Age: Bimodal distribution. First peak in the 3rd
decade and second peak after 50 years.
• Sex: Increased incidence in males.
• It presents as a generalised lymphadenopathy (more
than one group of lymph nodes are significantly
enlarged non-contiguous). Significant lymphadeno-
pathy refers to a lymph node of 2 cm size in the
groin, 1 cm size in nodes in the neck or axilla, firm to
hard in consistency, palpable left supraclavicular
node of any size.
• The disease starts in the left posterior triangle as a
group of lymph nodes with a ‘bunch of grapes’
appearances. This is seen in about 80% of cases.
• The nodes are firm (India rubber consistency) without
matting. In advanced cases and in poorly differentiated variety, matting can occur (Fig. 29.16).
• By means of contiguous and centripetal spread, other
lymph nodes in the neck, axillary, mediastinal, paraaortic and inguinal lymph nodes get enlarged (Fig. 29.17).
• Abdominal pain can occur due to hepatospleno-
megaly. The enlarged liver and spleen are smooth
and firm with round borders.
• Para-aortic nodes (Key Box 29.8) are felt in the
umbilical region, more so on the left side. Its clinical
features are:
Nodular, firm to hard mass, fixed mass
–
221
Fig. 29.16: Massive cervical lymph node enlargement. Observe
the scar of the lymph node biopsy on the opposite side, reported
as normal
Fig. 29.17: Advanced stage of Hodgkin’s lymphoma: Bilateral
massive enlargement of nodes
Key Box 29.8
Para-aortic Node Enlargement Common Causes
Lymphoma
Testicular tumours
Malignant melanoma
Gastrointestinal malignancy
– Does not move with respiration
– Being retroperitoneal, the mass does not fall
forward on knee-elbow position
– Pulsations may be felt over the mass (transmission
from aorta)
• Alcohol-induced pain in the involved lymph nodes
is a rare symptom.
Table 29.5 Hodgkin’s lymphoma (HL)
Classical HL Nodular lymphocytic predominant HL
1. Cells Large atypical cells (Reed-Sternberg cell) LPHL (popcorn cell—L and H cell)
2. Immunohistochemistry Express CD15 and CD30 (20 and 45 negative) Express CD20+, CD45+
3. Progress Slow (No ‘B’ symptoms, no bulky disease) Indolent, favourable prognosis
4. Spread Contiguous pattern of spread Does not have contiguous pattern of spread
5. Treatment Extended field Involved field RT only
6. Prognosis Good. Depends on staging Excellent
Section II • General Surgery
222
Manipal Manual of Surgery
• There may be ascites.
• Intermittent fever (irregular) is sometimes seen. Skin
rashes
1
are rare in Hodgkin’s lymphoma. Severe
pruritus without obvious skin lesions on clinical
examination may suggest occult Hodgkin’s
lymphoma. They may not respond to topical and
systemic agents and can be an early clue to the
presence of clinically occult HL23.
• Multiple bony pains may occur due to secondary
deposits, especially in the lumbar vertebrae. The
secondary deposits are usually osteoblastic giving
rise to ivory vertebrae.
• Superior vena caval obstruction indicates enlarged
mediastinal nodes. This is tested by asking the patient
to raise the hand above the head. Engorgement of
the veins indicates obstruction and the test is said to
be positive (Pemberton’s test).
Mediastinal Lymphoma (Key Box 29.9)
Key Box 29.9
Mediastinal Lymphoma
It is the most common malignancy in the mediastinum.
Anterior compartment is most commonly involved
(posterior—rare).
Most commonly used treatment is chemotherapy/
radiation.
Most dramatic response to the chemotherapy/radio-
therapy with a cure rate of 90% in early Hodgkin’s and
60% in advanced stage.
Even in patients with mediastinal bulky lymphoma,
symptoms of cough, chest pain, dyspnoea or SVC
obstruction is not common.
Mediastinal mass ratio (MMR), defined as the ratio of
maximum transverse diameter of mediastinal mass to
the maximum transverse intrathoracic diameter, must
be calculated. MMR greater than 0.33 by chest X-ray
or 0.35 by CT predicts a worse prognosis.
Generalised pruritus occurs in 25% of patients with Hodgkin’s
lymphoma. It may be the only presenting feature, preceding the
diagnosis by months.
Staging (Table 29.6)
Investigations
. Complete blood count (CBC): Peripheral smear to
1
rule out leukaemia. Anaemia indicates widespread
bone narrow metastases.
Table 29.6 Cotswolds revision of the Ann Arbor staging
system
Stage Description
I Involvement of a single lymph node region or lymph
node structure
II Involvement of two or more lymph node regions on
the same side of the diaphragm
III Involvement of lymph node regions or structures on
both sides of the diaphragm
IV Involvement of extranodal sites beyond “E” sites
Annotation description
A—No B symptoms
B—Fever, weight loss >10% over 6 months, or night sweats
E—Involvement of a single extranodal site contiguous or
proximal to known nodal site
X—Bulky disease as defined by >1/3 widening of mediastinum
at T5–T6, or >10 cm maximum dimension of nodal mass
2. Elevated creatinine and blood urea nitrogen (BUN)
indicate ureteral obstruction or direct involvement
of kidneys (increased uric acid levels indicate
aggressive non-Hodgkin’s lymphoma).
3. Alkaline phosphatase, calcium, and albumin levels.
First two are elevated, whereas albumin and haemoglobin levels are lowered.
4. Uric acid levels: Increased levels indicate tumour
burden or activity. It is associated with B symptoms.
Also, when the tumour responds to chemotherapy,
tumour lysis syndrome develops, and large amount
of uric acid is liberated which
may result in obstructive
nephropathy also.
5. Chest X-ray is taken to rule out mediastinal lymph
nodes, mediastinal widening, and/or pleural effusion.
6. Abdominal USG: To look for para-aortic nodes,
splenomegaly, secondaries in the liver or hepatomegaly.
• However, CT scan of the abdomen is better.
7. CT scan
• The investigation of choice for staging. CT scans
of the chest, abdomen, and pelvis are mandatory.
CT of the neck is optional.
• CT to define para-aortic nodes when there is
minimal enlargement (≥0.5 cm).
• To calculate MMR (see mediastinal lymphoma).
• To assess renal function and detect back pressure
hydronephrosis.
8. PET scan: It is better investigatory modality today.
1
Mycosis fungoides: It is not a fungal infection but is caused by non-Hodgkin’s lymphoma with infiltration of the skin with malignant lymphocytes.
Dermatitis and papular rashes are common which progress to tumour formation. It is a variety of cutaneous T cell lymphoma.
Section II • General Surgery
Lymphatics, Lymph Vessels and Lymphoma
223
9. Lymph node biopsy: Incision biopsy is done and a
neck node is usually removed. Fine needle aspiration
cytology (FNAC) may give the diagnosis but a
definite histological pattern cannot be made out by
FNAC. A trucut biopsy may also give the diagnosis.
10. Mediastinoscopy (Chamberlain procedure) is done
if peripheral nodes are not available.
TREATMENT OF HODGKIN’S LYMPHOMA
• Chemotherapy has made a great revolution in the
treatment of lymphoma. It is given in almost all
stages. Radiotherapy is given in early stage 1cases as
involved field therapy protecting the surrounding
structures such as abdomen.
• There are several regimens for the treatment of
Hodgkin’s lymphoma. Students are advised to refer
medical oncology books, if necessary. However, a few
commonly used regimens are given below. Please
note: MOPP regimen is no longer followed because
of toxicity.
Regimen 1
ABVD: Adriamycin Bleomycin Vinblastine Doxorubicin.
4 to 6 Cycles has
become the standard chemotherapy
regimen (details are given below). Doxorubicin 25 mg/
2
IV, days 1 and 5, Bleomycin 10 mg/m2 IV, days 1
m
and 5, Vinblastine 6 mg/m
Dacarbazine 375 mg/m
2
2
IV, days 1 and 5 and
IV, days 1 and 5.
Early Complications of ABVD Regimen
• Adriamycin: Acute cardiac toxicity.
• Bleomycin: Pulmonary toxicity.
• Vinca alkaloids: Nausea, vomiting, tumour, neutro-
paenia, peripheral neuropathy.
• DTIC: Hair loss.
Regimen 3
BEACOPP: Bleomycin, etoposide, doxorubicin, cyclo-
phosphamide, vincristine, procarbazine and prednisone.
STAGEWISE TREATMENT OF HODGKIN’S LYMPHOMA
Stage 1 and stage 2
• Chemotherapy followed by involved site radio-
therapy if the disease is favourable. It means the
disease is not bulky, disease is in less than 3 different
lymph node sites on one side of diaphragm without
any B symptoms and ESR is not elevated.
• Otherwise, if these criteria mentioned above are
present, treatment remains the same with or without
addition of immunotherapy using brentuximab
vedotin or nivolumab, etc.
• Consider bone marrow transplant also.
Extended mantle field radiotherapy may cause carcinoma of
the breast. Pelvic radiotherapy (inverted Y) may cause
infertility. Hence, they are not commonly used now.
Stage 3 and stage 4
The treatment remains the same except radiotherapy.
Different varieties of chemotherapy are given not only
ABVD but other regimens such as BEACOPP, immuno-
therapy and stem cell transplant.
Complications of Disease (Natural History)
1. Mediastinal disease—pleural effusion, superior vena
caval obstruction
. HL decreases cell-mediated immunity. It causes
2
depressed CD4+/CD8+ ratio. These patients are vulnerable for opportunistic infections—mycobacteria,
herpes zoster, cytomegalovirus (Key Box 29.10).
Late Complications of ABVD Regimen
• Life-threatening cardiovascular disease: This is due
to mediastinal radiation—pericarditis, cardiomyopathy, cardiac failure.
• Development of second cancer: Acute leukaemia:
Incidence is less now as MOPP regimen is rarely used.
Lung cancer, breast cancer, melanoma, soft tissue
sarcoma, thyroid cancer—may develop after 30 years.
• Hypothyroidism and hyperthyroidism (Graves’
disease).
Regimen 2
AAVD: Bleomycin is replaced by brentuximab vedotin.
AAVD is given every 2 weeks for 6 months. Brentuximab
vedotin is an antibody-drug conjugate. Ideally patients
who have CD 30 positive respond well to this drug as it
delivers chemotherapy only to these cells.
Key Box 29.10
Advanced HL—Independent Prognostic Factors
Albumin less than 4 g/dl
Lymphocytopaenia <600/mm
Blood Hb less than 10.5 g/dl
Under 44 years of age
Male sex
Involvement of liver, bone (advanced)
Neutrophils (WBC) >15,000 cells/mm
Remember as ALBUMIN
3
3
, leukocytosis
What is no Longer followed in Hodgkin’s Lymphoma?
1. Staging laparotomy: With the availability of CT,
ultrasound, and lymphoscintigraphy, all details of
intra-abdominal visceral involvement or pathology
can be detected.
Section II • General Surgery