Добавил:
Sekretar
kiopkiopkiop18@yandex.ru
t.me/Prokururor I Вовсе не секретарь, но почту проверяю
Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз:
Предмет:
Файл:Ординатура / Хирургия / @xirurgi_2025 / @xirurgi_2025 - 191 - файл
.pdf
246
https://t.me/medicina_free
SRB’s Manual of Surgery
Lipodystrophy and lipoidosis (lipoedema). Lipoedema
occurs exclusively in females; begins in puberty; bilateral and
symmetrical; trunk may be involved; feet are not involved;
not pitting; not related to elevation/compression; MRI shows
only fat without fluid.
Arterial diseases including AV malformations.
Gigantism.
Drug induced— steroids, estrogens, nifedipine.
External compression of veins as caused by abdominal tumours.
INVESTIGATIONS
B
For the cause, venous Doppler, US abdomen
ESR, peripheral smear.
Lymphangiography, Isotope-lymphoscintigraphy.
MRI, CT scan to identify the cause.
Lympho fluruoscopies using indocyanine green is excellent method
Complications
Skin thickening, abscess and maggot’s formation.
Recurrent cellulitis, nonhealing ulcers, septicaemia.
Lymphangiosarcoma/Stewart Treves syndrome (0.5%,
occurs after 10 years), which presents as multiple bluish
satellite nodules in the skin of the limb often with ulcera-
tion and haemorrhage. Skin/nodule biopsy is confirmative.
Chemotherapy, radiotherapy, later even though radical
amputation is the treatment, it carries very poor prognosis.
This syndrome is usually seen in upper limb after mastectomy.
Recurrent streptococcal infection.
Treatment
Conservative
Elevation of the limb, exercise, weight reduction.
Static isometric activities like prolonged standing or carrying
weights should be avoided; rhythmic isotonic movements like
swimming/massaging should be encouraged.
Diuretics to reduce the oedema is controversial. It more often
causes eletrolyte imbalance than being beneficial.
Benzopyrones are protienolytic agents/lympedim. They are
coumarin (I, 2 benzopyrones) derivatives with no anticoagu-
lant effect but increase the lymphatic peristalsis and pumping
mechanism along with proteolysis.
Daily wearing of below knee stockings. It reduces the oedema,
skin changes, lymphoerrhoea; softens and supports the
inelastic skin.; may restore the shape of the limb. ABPI should
be assessed prior using the MLLB.
Avoid trauma and infection.
ntermittent pneumatic compression devices (Pressure
>50 mmHg); multilayered lymphoedema bandaging
(MLLB)—nonelastic type is preferred method; graded stock-
ings. It reduces the oedema, skin changes, lymphoerrhoea;
softens and supports the inelastic skin; may restore the shape
of the limb. ABPI should be assessed prior using the MLLB.
Antibiotics—flucloxacillin, erythromycin, long-acting penicillins.
Topical antifungal 1% clotrimazole and systemic griseofulvin
250–1000 mg.
Regular washing and keeping the limb clean is very important.
Diethyl carbamazine citrate (DEC) 100 mg TID for 3 weeks.
Pain relief—by suitable means. NSAIDs are better avoided
in lymohoedema as it may precipitate necrotizing fasciitis.
Skin care:
¾
Keratolytics like salicylic acid 5%; bland emollient; soft/
liquid paraffin.
¾
Avoidance of skin sensitisers like some soaps.
¾
Topical steroids.
¾
Control of allergy.
¾
Control of fungal infection by drugs like fluconazole.
¾
Three per cent benzoic acid ointment to prevent Athlete’s
foot.
¾
Control of lymphorrhoea.
¾
Prevention/control of skin infections.
Complex decongestive therapy is a comprehensive two
phase program of elevation, exercise, massaging, and
compression wraps. First phase is intensive therapy and
second phase is maintenance therapy.
Compression wraps may be high stretch wraps or low
stretch wraps. Low stretch wraps are better accepted. It
should be worn initially for 24 hours. Compression wraps are
used in initial intensive phase of therapy. Graduated elastic
compression garments are used in maintenance phase which
provides maximum pressure of 50 mmHg at the distal part
with gradual reduction of pressure in proximal portion.
Manual lymphatic drainage is a specialised technique to stimulate
the contractility of lymph collecting vessels and enhance fluid
and protein transport by gentle, light, superficial massaging of
the skin so as to open up new lymphatic vessels. Technique is
done first on the opposite normal side; then trunk, same side
trunk; same side proximal; same side distal and later same side
distal to proximal fashion, so as to redirect the lymph towards
functioning lymphatic territories.
Surgery
Surgeries for lymphoedema has been classified as:
Excisional
¾
Charle’s operation.
¾
Homan’s operation.
Physiological
¾
Omentoplasty.
¾
Nodovenous shunt (Neibulowitz).
¾
Lymphovenous shunt (O’Brien’s).
¾
Ileal mucosal patch.
Here either communication between superficial and deep
lymphatics are created or new lymphatic channels are
mobilised to the site.
Omentoplasty (Omental pedicle): As omentum contains
plenty of lymphatics, omental transfer with pedicle will
facilitate lymph drainage.
Combined: Both excision + creation of communication
between superficial and deep lymphatics.
¾
Sistrunk operation.
¾
Thompson’s operation.
¾
Kondolean’s operation.
Fig. 1.4 4 6: Charle's excisional surgery. Here after excising
https://t.me/medicina_free
lymphoedematous tissue, area is covered with skin graft.
247
CHAPTER 1O General Surgery: Lymphatics
Fig. 1.4 48: Diagram showing right side groin nodovenous shunt
between inguinal lymph node and long saphenous vein. Left side
showing lymphovenous shunts between dilated lymphatics and long
saphenous vein. At least 4 lymphatics should be anastomosed using 7
zero/11 zero prolene—using operative microscope.
Fig. 1.447: Thompson’s Swiss-roll operation. Here after removal of
lymphoedematous tissue, deep fascia is opened to expose the muscle.
Epidermis abraded using skin graft knife. This shaved dermis is buried
into the muscle to get communication into the deeper lymphatics.
Bypass procedure:
¾
Handley’s (1908) silk threads/nylon threads/perforated
polyethylene tubes placement as burial from ankle to
mid-abdominal level, kept for one year. Procedure is only
of historical interest.
¾
Skin bridge across the thigh and abdomen (Gillies).
¾
Nodovenous shunt.
¾
Lymphovenous shunt using microscope.
¾
Ileal mucosal patch (Kinmonth). Segment of ileum with
pedicle is isolated and opened to expose the mucosa;
mucosa is denuded and this mucosa is placed in the thigh
as burial to communicate with lymphatics to drain into
abdominal lymphatics across ileum.
¾
Baumeister lymphatic grafting.
¾
Autotransplantation of free lymphatic flap from opposite
side—done in post-mastectomy lymphoedema (Trevidic
and Cormier).
Limb reduction surgeries:
¾
Sistrunk operation: Along with excision of lymphoe-
dematous tissue, window cuts in deep fascia is done, so
as to allow communication into normal deep lymphatics.
¾
Homan’s operation: Excision of lymphoedematous
tissue is done after raising skin flaps. Later skin flaps are
Fig. 1.4 4 9: Believe it or not! Severe scrotal lymphoedema reaching
almost up to feet; after surgical excision it weighed 40 kg; postoperatively patient went home with – 40 kg weight (Courtesy: Professor
Shivananda Prabhu, MS, KMC, Mangaluru).
trimmed to required size and sutured primarily. Medial
and lateral sides of the limb are done at separate sittings
with 6 months interval.
¾
Thompson’s operation: Lymphoedematous tissue is
excised under the skin flaps. Epidermis and part of
the dermis of one of the skin flaps is shaved off using
Humby’s knife. It is buried under opposite flap, deep to
See no evil; hear no evil; speak no evil.
248
https://t.me/medicina_free
the deep fascia like a swiss roll (Swiss roll operation or
buried dermal flap operation).
Problems here are formation of epidermal cysts and sinus.
¾
Kondolean’s operation: Along with excision of lymphoe-
dematous tissue, vertical strips of deep fascia is removed
so as to open the deep lymphatics which creates communication between superficial and deep lymphatics.
¾
Macey’s operation: Here skin and subcutaneous tissue
are peeled back with deep fascia and split skin grafting
is done over the denuded area. Overlying pad of tissue is
sutured back temporarily and after 10 days, it is trimmed
away.
¾
SRB’s Manual of Surgery
Miller’s procedure: It is excision of subcutaneous tissues
under the skin flap with deep fascia in two stages. First
stage is done over the medial aspect of the limb; second
stage done after two months over lateral aspect of the
Fig. 1.450: Lymphoma involving neck nodes. Differential diagnosis
could be tuberculous lymphadenitis.
limb.
¾
Charle’s (1912) operation: Done in severe lymphoedema
with elephantiasis. Along with excision of lymphoedematous tissue, skin grafting is done. It reduces the size and
weight of the limb. Patient becomes ambulatory. Wound
sepsis, graft failure, dermatitis, hyperkeratosis are the
complications.
¾
Reduction surgeries are done for lymphoedema of
scrotum, penis, labia and eyelid.
In severe type, occasionally amputation may be required.
THERAPEUTIC STRATEGY FOR LYMPHOEDEMA
B
Evaluation
Skin care; limb care
Pain control
Management of swelling
Drugs
Exercise
Surgery
LYMPHOMAS
They are progressive neoplastic condition of lymphoreticular
system arising from stem cells. They are heterogeneous group
of lymphoproliferative malignancies results from clonal expansion of tumor cells derived from B, T or NK cells. 80–90% is
derived from B cells.
Lymphomas are the 3rd most common malignancy among
children comprising 15% of paediatric cancers.
Aetiology
Genetic predisposition.
Sjogren’s syndrome—30 fold increase of NHL.
HIV infection.
Wiskott—Aldrich syndrome.
Ataxia—telangiectasia.
Bloom’s syndrome.
Virus aetiology—Epstein-Barr virus.
Celiac sprue—intestinal T cell lymphoma.
H. pylori may be associated with MALT lymphoma.
Occupation causes—hair dye workers; herbicide exposure.
Ionising radiation.
A
B
Figs. 1.451A and B: Stage IV lymphoma with neck nodes/sternal
swelling/axillary nodes which has ulcerated (ulceration is not common
in lymphoma).
Smoking; alcohol consumption; tobacco usage.
Lymphomas are more common in western countries than
in Asia.
Types: (a) Hodgkin’s lymphoma (HL); (b) Non-Hodgkin’s
lymphoma (NHL).
WHO modified REAL classification (Revised European American
https://t.me/medicina_free
Lymphoma) of lymphoma:
B-cell neoplasms
– Precursor B cell neoplasm—ALL, LBL.
– Peripheral B cell neoplasm—it includes all B cell related non-
Hodgkin’s lymphomas
T-cell and putative NK cell neoplasms
– Precursor T cell neoplasms—ALL and LBL T cell related
– Peripheral T cell and NK cell neoplasm—it includes all T cell
related non-Hodgkin’s lymphomas
Hodgkin’s lymphoma
– Predominant HL—nodular lymphocyte type
– Classical HL
- Nodular sclerosis
- Lymphocyte rich
- Mixed cellularity
- Lymphocyte depletion
Precursor lymphoid neoplasms.
Immunodeficiency-associated lymphoproliferative disorders.
Note: ALL is acute lymphoblastic leukaemia. LBL is lymphoblastic
lymphoma.
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, usually discrete (without matting), nontender,
typically with India rubber consistency.
Pain after alcohol consumption even though is very
uncommon (3%) with low sensitivity but when present
has got high specificity and is pathognomonic of HL. Pain
appears within 3 minutes after alcohol intake in the vicinity
of the involved node.
Site: Cervical lymph nodes most common—82% (lower deep
cervical group and in posterior triangle). Others include axillary,
mediastinal, inguinal, abdominal. Axial lymphatics are commonly
involved.
Specic Features
249
CHAPTER 1O General Surgery: Lymphatics
OLDER CLASSIFICATIONS (NOT PRACTICED NOW)
B
Jackson and Parker (1944): Paragranuloma; granuloma; sarcoma.
Lukes and Butler (1966): Lymphocytic and histiocytic nodular;
lymphocytic and histiocytic diffuse; nodular sclerosis; mixed
cellularity; diffuse fibrosis; reticular.
HODGKIN’S LYMPHOMA (HL)
(The lymph glands of the neck) exhibited a firm cartilaginous
structure of a light colour and very feeble vascularity, but with no
appearance of softening or suppuration. Glands similarly affected
accompanied the vessels into the chest, where the bronchial and
mediastinal glands were in the same state and greatly enlarged….
The spleen was enlarged to at least four times its natural size…
presenting the same structure as the enlarged glands.
—Thomas Hodgkin, 1832
It is the most common type of lymphoma.
Infectious mononucleosis, Epstein-Barr virus, HIV infection and
genetic monoclonal B cell disorder (90%) may be the aetiologies.
Grossly lymph nodes are fleshy, pinkish grey, and rubbery
in consistency.
Microscopically contains cellular infiltration with lymphocytes,
reticulum cells, histiocytes, fibrous tissue and Reed-Sternberg
cells: (Reed-Sternberg cells are giant cells with two large mirror
image nuclei). Owl eye, 50 µm sized. Reed Sternberg cell is a
“crippled” germinal center B cell which does not have normal B
cell surface antigens; micromanipulation of single cell followed
by PCR shows clonally rearranged, nonfunctional IG genes.
RYE CLASSIFICATION
B
Lymphocytic predominance. Has got good prognosis—rare
Mixed cellularity—common, associated with EBV.
Nodular sclerosis—commonest.
Lymphocytic depletion. Has got poor prognosis—rare.
(Reed-Sternberg cells are also seen occasionally in certain other
conditions like glandular fever).
Nodular sclerosis is most common type.
Consecutive group of lymph nodes are involved.
Splenomegaly is very common (45%).
Hepatomegaly with jaundice (5%)—jaundice is due to
haemolysis or due to diffuse liver involvement.
Para-aortic lymph nodes may be enlarged and often palpable
as vertically placed mass at or just left of the midline, which
does not move with respiration, does not fall forward,
nonmobile, resonant, smooth, firm mass often with trans-
mitted pulsation from aorta (secondaries are hard, nodular
usually primary from GI, melanoma, testis). Ascites is not a
common feature.
Pruritus—25%. It may be the only presenting symptom. It
is usually seen in nodular sclerosis type.
Constitutional symptoms like fever, night sweats, 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
B
Weight loss, more than 10% in 6 months.
Fever, (earlier called as Pel Ebstein fever is cyclical high fever,
actually is due to brucellosis).
Night sweats.
Mediastinal lymph node involvement may cause compres-
sion features like SVC obstruction. Mediastinal lymphoma
is the most common mediastinal malignancy which usually
occurs in anterior mediastinum. Occasionally presentation
may be difficulty in breathing, chest pain, dysphagia and
SVC obstruction (Pemberton’s sign may be positive). It may
be asymptomatic also. If ratio between maximum trans-
verse diameter of mediastinal mass to maximum transverse
intrathoracic diameter (MMR) is more than 0.33 in chest
The difference between ordinary and extraordinary is that little extra.—Zig Ziglar
250
https://t.me/medicina_free
SRB’s Manual of Surgery
A
B
Figs. 1.452A and B: Lymphoma in a boy and in an adolescent with
neck nodes. Note the diffuse nature. Nodes are usually India rubber
consistency.
X-ray or more than 0.35 in CT chest, then it carries worst
prognosis.
Occasionally bone like vertebrae may get involved.
Anaemia, pancytopenia, fatigue, bone pain, petechiae with
red coloured skin patches, nephrotic syndrome (minimal
change disease).
Differential diagnosis:
Tuberculosis; NHL; HIV; Chronic
lymphatic leukaemia; Nonspecific lymphadenitis; Sarcoidosis;
Secondaries in lymph nodes.
ANN-ARBOR CLINICAL STAGING
B
Stage 1: Confined to one group of lymph node
Stage 2:
Stage 3:
Stage 4:
Suffix ‘S’—Spleen involved
Suffix ‘B’—Presence of constitutional symptoms
Suffix ‘A’—Absence of constitutional symptoms
Note: In modification, following additions are there:
N—Nodes
M—Marrow
Stage III (1) is nodes above renal vein level and
Stage III (2) is below it.
In Cotswolds revision of Ann Arbor classification:
Stage 3: Involvement of regions or structures on both sides of the
diaphragm.
Stage 4: Involvement of extranodal sites beyond E sites. Here E
means—involvement of single extranodal site contiguous or proximal
to known nodal site.
X means: Bulky disease which is defined as >1/3rd widening of mediastinum at T5–T6 or >10 cm maximum dimension of nodal mass.
More than one group of lymph nodes on one side of the
diaphragm
Nodes involved on both sides of the diaphragm
Extranodal involvement like liver, bone marrow
Single extralymphoid site is I E
An extralymphoid site with one or more lymph nodes same side
of diaphragm is II E
An extralymphoid site with lymph nodes on both sides of
diaphragm III E.
An extralymphoid site with spleen and lymph nodes on both sides
of diaphragm III SE.
Spleen with lymph nodes on both sides of diaphragm is III S.
H—Liver S—Spleen L—Lung
P—Pleura O—Bone D—Skin
Fig. 1.453: Hodgkin’s lymphoma in neck region.
Figs. 1.454A and B: Fungating lymphoma in both axilla of a patient.
BA
Fig. 1.455: Secondaries in neck lymph nodes. It is hard, large
probably fixed and nonmobile making it advanced and inoperable.
Investigations
https://t.me/medicina_free
Blood: Hb%, ESR, peripheral smear, blood urea, serum creati-
nine. Serum alkaline phosphatase and calcium may elevated.
FNAC of lymph nodes. Not very sensitive; but initially done
to rule out secondaries or tuberculosis.
Excision biopsy of lymph nodes. Entire lymph node is excised
to retain the architecture of the lymph node. It is important to
grade the tumour. It is better to have immunohistochemistry of
tumour tissue. As cell mediated immunity is decreased, CD4/
CD 8 ratio will be decreased. CD 15, 30, 45 are very useful.
Chest X-ray—to look for mediastinal lymph nodes, pleural
effusion.
Ultrasound abdomen—to look for the involvement of liver,
spleen, abdominal lymph nodes.
CT scan of mediastinum/chest, abdomen and pelvis is better,
ideal and essential. CT is used ideally to stage the disease.
251
CHAPTER 1O General Surgery: Lymphatics
Fig. 1.457: Staging laparotomy for Hodgkin’s lymphoma. Needle and
wedge biopsies from liver/nodal biopsies from para-aortic, celiac,
mesenteric, iliac nodes/splenectomy/ovarian translocation/iliac crest
biopsy are the components of staging laparotomy. This can be very
well-achieved through laparoscopy also now. Staging laparotomy is
not commonly done now.
A
B
Figs. 1.456A and B: Mediastinal nodes involved in lymphoma as
seen in chest X-ray and chest CT scan.
MRI and PET scan are very useful to identify extranodal tissue
involvement. Gallium scan is less useful.
Bone marrow biopsy or aspiration to stage the disease and
to see the response to treatment. Usually iliac crest biopsy is
done under local anaesthesia. It gives the staging.
Chamberlain’s mediastinoscopy and biopsy of mediastinal
lymph node is done if peripheral nodes are not available for
biopsy. Laparoscopy and biopsy of different abdominal lymph
nodes is also a good option.
Lower limb lymphangiography to look for the pelvic and retroperito-
neal lymph nodes. It shows reticular pattern in node; it can be used
to assess the therapeutic response and prognosis. Lymphoscintigraphy is better and acceptable. It is prognostic tool also.
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 para-aortic, celiac
mesenteric, iliac nodes. In females, ovaries are fixed behind
the uterus to prevent radiation oophoritis (oophoropexy/
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.
– It is done in stage I/IIA lymphoma (HL) in selected patients.
Note:
Staging laparotomy, splenectomy (requires for immune function),
lymphangiogram, IVU are no longer/very rarely done now for HL.
PROBLEMS IN HL
B
Pleural effusion—respiratory discomfort
SVC obstruction
Spine if involved—not very common but can occur
Opportunistic infection—mycobacteria, cytomegalovirus, herpes
zoster
Bronchopneumonia, septicaemia
Immunosuppression and its effects
Risk for other malignancies in later life
Man cannot discover new oceans unless he has courage to lose sight of the shore—Anonymous
252
https://t.me/medicina_free
Differences between HL and NHL
T
HL (more common) NHL
Age: Young and elderly Middle age and elderly
Pattern of
involvement:
Cervical lymph
node:
Splenomegaly: Common Not common
Peripheral
lymph node
involvement
SRB’s Manual of Surgery
(e.g.
epitrochlear
nodes)
Treatment: Mainly radiotherapy
Prognosis: Better Poor
Symmetrical and
consecutive
Commonly involved Any group can be
Not common Common
Chemotherapy (ABVD
regime)
Asymmetrical
involved
Mainly chemotherapy
Treatment for HL
Stage I and II:
¾
Mainly radiotherapy—external high cobalt RT.
–
Above the diaphragm—“Y” field therapy, covering
cervical, axillary, mediastinal lymph nodes. It may
cause carcinoma of breast.
–
Below the diaphragm, mantle or inverted “Y” field
therapy, covering para-aortic and iliac nodes. It may
cause infertility.
¾
Chemotherapy is also given.
Stage III and IV: Mainly chemotherapy.
Treatment for Relapses
Autologous stem cell transplantation.
High dose chemotherapy.
High dose chemotherapy with autologous stem cell trans-
plantation.
MOPP/ABV hybrid regime.
Single dose vinorelbine (new vinca alkaloid); gemcitabine;
immunotherapy; tumour vaccination; gene therapy.
TREATMENT STRATEGY FOR HL
B
Early favourable stage—extended field radiation only
Early unfavourable stage—extended radiation plus chemotherapy
Advanced stage—extensive chemotherapy often with local radiation
UNFAVOURABLE SIGNS IN EARLY HL
B
1. Large mediastinal mass
Extranodal disease
2.
3.
Elevated ESR
Four or more involved regions
4.
Presence of B symptoms
5.
Note:
First five parameters are most
important.
Chemotherapy for Hodgkin's Lymphoma (HL)
T
Mopp regime (1960) Not used
now
Mustine (Mechloroethamine)—6
mg/q meter on 1st and 8th day.
Oncovin (Vinca alkaloids)—1.4
mg/sq meter on 1st and 10th day
Procarbazine—100 mg orally
daily for 10 days
Prednisolone—45 mg orally daily
for 10 days
Note:
MOPP is no longer considered
effective regime
Note: Gonadal dysfunction and growth retardation is the long-term adverse effects in childhood HL due to treatment (RT and CT).
6. Anaemia <10 g% low serum
albumin level <4 g%
7.
Age more than 50
Male gender
8.
ABVD regime (Standard) Stanford V regime BEACOPP regime
Adriamycin—30 mg/m
(cardiotoxic)
Bleomycin—10 mg/m
(pulmonary fibrosis)
Vinblastine— 6 mg/m
marrow suppression)
DTIC/Dacarbazine 350 mg/m
Note:
ABVD is becoming more
popular, standard and
commonly used regime now. It
started in Italy, 1970.
It is commonly used in US.
2
2
2
(bone
meter
Prognosis
Stage I and II—80%.
Stage III A—70%.
Stage III B and stage IV—<40%.
PROGNOSTIC FACTORS
B
Stage I and II has got better prognosis
Lymphocytic predominance has got better prognosis
Stage “A” without constitutional symptoms has got better
prognosis
Anaemia <10 g%; hypoalbuminaemia <4 g%; lymphocyte count
<600/mm
of bone and liver are other poor prognostic factors
Adriamycin (Doxorubicin)
Bleomycin
Vincristine, vinblastine
Mechlorethamine
Etoposide
Prednisolone
2
Note:
It involves more intensive
schedule with incorporation of
radiotherapy.
Not well accepted.
3
; WBC count >15,000/mm3; age >50 years; involvement
Bleomycin
Etoposide
Adriamycin (Doxorubicin)
Cyclophosphamide
Oncovin – Vincristine
Procarbazine
Prednisolone
Note:
It is commonly used in
Europe; its efficacy is 15%
better than ABVD; but it is
costly; it needs granulocyte
stimulating factor to control
adverse effect; secondary
leukaemia is slightly higher
here
NON-HODGKIN’S LYMPHOMA (NHL)
https://t.me/medicina_free
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. (40%).
Secondary infection, cachexia and immunosuppression is
more common.
Inner Waldeyer ring and oropharynx lymphoma (NHL-B cell)
may be associated with MALT lymphoma of stomach; so
gastroscopy is indicated in these patients.
Pyoderma gangrenosum may the presentation.
Small bowel lymphomas are usually NHL type. Western
type is annular, ulcerative multiple B cell type, presents as
obstruction, bleeding, weight and appetite loss, perforation.
Celiac disease related lymphoma is usually primary T cell type
presents with severe unresponsive diarrhoea, PUO, obstructive features. Mediterranean lymphoma of small bowel is seen
in North America and Middle East which is associated with
alpha chain disease.
Sarcoma, carcinomas/secondaries are the differential diag-
nosis for NHL.
TYPES
B
Nodular (Follicular)
Diffuse lymphocytic
Rappaport classification Working classification
• Nodular • Low grade
• Diffuse • Intermediate grade
Undifferentiated
Histiocytic type
• High grade
PATHOLOGICAL CLASSIFICATION
B
B cell type— Diffuse large B cell, small lymphocytic, follicular,
Burkitt’s lymphoma.
T cell type (10%)—cutaneous, mycosis fungoides, Sezzary
syndrome, lymphoblastic lymphoma.
Note:
Many different classifications are there for NHL. Students have to refer
pathology book when needed.
Histochemistry and tumour markers are very sensitive and
essential tools other than biopsy; CT chest, abdomen, pelvis; MRI
spine, peripheral smear and bone marrow biopsy.
Serum LDH, calcium, CD5, CD23 counts are very useful prog-
nostic indicators. Raised LDH carries poor prognosis and also
patients require aggressive chemotherapy.
Treatment
Mainly chemotherapy
Various regimens available include:
CHOP regime is used–Cyclophosphamide; Hydroxydauno-
rubicin; Oncovin; Prednisolone. This is the standard regime
commonly used.
¾
R CHOP regime is adding Rituximab when lymphoma is
B cell type.
¾
R-CHOEP regime is when Etoposide is added.
¾
Side effects are–haemorrhagic cystitis (Mesna is given);
alopecia; nausea and vomiting; neutropenia; severe
sepsis.
ABVD—Adriamycin, Bleomycin, Vincristine, Dacar-bazine.
ABVP—Adriamycin, Bleomycin, Vincristine, Prednisolone.
Combinations of above.
Rituximab may be used with chemotherapy regimes.
Role of radiotherapy in NHL: When vertebra is involved.
Prognosis is poor compared to HL.
Note:
• Diffuse large B cell (DLBCL) type is the most common type of NHL
(30–40% of cases); 50% of patients have organ involvement at the
time of diagnosis. Subtypes are—Primary mediastinal B cell lymphoma
(PMBL); Primary CNS lymphoma; EBV-positive DLBCL; T-cell/histiocyterich large B cell lymphoma, etc.
253
CHAPTER 1O General Surgery: Lymphatics
Regime and drugs for NHL
T
Drugs Standard R-CHOP 14 or
Rituximab 375 mg/m
Cyclophosphamide 750 mg/m
Hydroxydaunorubicin 50 mg/m
Oncovin 1.4 mg/m
Prednisolone 40 mg/m
R – Maxi – CHOP is used in Mantle cell lymphoma as 21 days interval regime alternating with R-HDAC (Rituximab with high dose cytarabine).
In other NHL excluding aggressive forms, R-CHOP standard dose is first line therapy.
R-CHOP 21 regime
2
2
2
2
2
Laugh at obstacles and cry at your success.
R-Maxi—CHOP regime Mode of administration Number of days
375 mg/m
1200 mg/m
75 mg/m
2 mg (max dose) Intravenous infusion Day 1
100 mg/m2 Orally qid Days 1–5
2
2
2
Intravenous infusion Day 1
Intravenous infusion Day 1
Intravenous infusion Day 1
254
https://t.me/medicina_free
• Bulky disease is defines as—Maximal diameter >7 cm in transverse or
coronal planes (MSKCC).
• International Prognostic Index: Based on 5 variables—(1) Age >60
years;(2)StageIII,IV;(3)Performancestatus:ECOG≥2;(4)Number
ofextranodalsites≥2;(5)LDH.
• Grey zone lymphoma is rare; has got features of both HL and DLBCL.
• PETCT scan is used to assess remission status.
• Prognosis may be favourable (>90% relapse free); intermediate (50 –
90% relapse free); unfavourable (<50% relapse free).
• Rituximab is monoclonal antibody injected either subcutaneously or IV
along with hyaluronidase.
• Targeted therapies becoming more popular in relapse cases; but they
SRB’s Manual of Surgery
are costly. Nivolumab (human Ig-G4 monoclonal antibody targeting the
programmed death-1 (PD-1) receptor); ibrutinib (Bruton’s tyrosine kinase
inhibitor); Chimeric antigen receptor gene therapy are different agents.
• NHL can be aggressive with short natural history, very aggressive or
indolent (35%) with long natural history.
• In HL cure rate is higher; stage is more important than histology;
response to therapy is higher. In NHL histology is more important tan
stage; cure rate is lower; response to therapy is not good.
MANTLE CELL LYMPHOMA
It is a type of NHL under B cell subtype comprising 6% of
NHL. It is aggressive type of NHL. MCL develops in the outer
edge of a lymph node called the mantle zone.
It is due to CD5 positive antigen-naive pre-germinal center B
cell within the mantle zone that surrounds normal germinal
center follicles. MCL cells generally over-express cyclin D1.
It is an acquired genetic disorder; MCL is neither communi-
cable nor inheritable.
Types are—nodular or diffuse pattern with two main cytologic
variants: typical or blastic (aggressive).
Common in old people; presenting with lymphadenopathy,
fever, weight loss, night sweats, splenomegaly often other
visceral organ involvement.
Investigations are – lymph node biopsy with immunohisto-
chemistry (CD5; CD10; CD23; cyclin D1); CT chest, abdomen,
pelvis; bone marrow biopsy; PET scan; cell proliferation index
(Ki-67). The Mantle Cell Lymphoma International Prognostic
Index (MIPI) was derived to classify as low, intermediate and
high-risk group patients. Beta-2 microglobulin is another risk
factor in MCL used primarily for transplant patients.
Treatment: Chemotherapy, immune-based therapy, radioim-
munotherapy and new biologic agents are used. R Maxi CHOP
alternating with R-HDAC is used as chemotherapy (see table
below). Fludarabine is also can be used additionally. Total
body irradiation with stem cell transplantation is also useful.
Immunotherapy using rituximab is used in combination; it
may be combined with radioactive molecules to have radioimmunotherapy. Targeted therapy using ibrutinib, temsirolimus,
bortezomib, also used.
Prognosis: MCL carries poor prognosis. Mean survival is 3
years.
MALT LYMPHOMA (MALTOMA)
It is lymphoma arising from mucosa associated lymphoid
tissue; it is actually extranodal marginal zone B cell lymphoma
of gut mucosa associated lymphoid tissue. It is usually
primary GI lymphoma (4% of gastric lymphoma); of nonHodgkin’s B cell type.
Commonest site is stomach. Helicobacter pylori infection is
the causative agent commonly.
It can be low grade or high grade.
¾
Low grade occurs in chronic gastritis patient with
Helicobacter infection. Diffuse mucosal thickening with
ulceration is seen. Anti-helicobacter therapy is very useful
in this type.
¾
High grade is aggressive one. It presents with features
similar to gastric carcinoma often with smooth, firm
gastric mass in epigastric region.
Dawson’s criteria for primary GI lymphoma (NHL)—No
palpable superficial lymph nodes; no thoracic lymph nodes in
CT scan; normal WBC count and bone marrow; predominant
bowel pathology; liver, spleen are normal.
Systemic features like fever, night sweats, and weight loss
can occur in 50% cases. Bleeding with haematemesis with
obstruction is not uncommon. Locoregional lymph nodes
(only) may get involved.
Endoscopic biopsy is essential to diagnose. Endosonog-
raphy, bone marrow biopsy, CT scan of chest, abdomen
and pelvis is a must to confirm primary GI/gastric
lymphoma and to identify or to rule out extragastric lymph
node NHL.
Ann Arbor staging system is commonly used but is inad-
equate. Lugano staging system is widely accepted one—
Early: Single primary lesion or multiple noncontiguous
primary lesions confined to GI tract with or without nodal
involvement; Advanced: Disseminated extranodal spread or
supradiaphragmatic spread.
Treatment: First line treatment— Primary chemotherapy with
anti-Helicobacter pylori regime, often with RT is the standard
treatment. Palliative gastrectomy in case of bleeding, perforation and obstruction is done. Radical gastrectomy is done in
diseases which are confined to stomach only to in selected
individuals (limited role).
¾
Early stage (Lugano) with +ve H. pylori—H. pylori eradi-
cation therapy is used; Early stage with – ve H. pylori or
with H. pylori therapy failure, local RT is used often with
chemotherapy as single or multiple agents.
¾
Advanced stage (Lugano) is treated with H. pylori regime
with immunotherapy (Rituximab with fludarabine) and
chemotherapy (oral cyclophosphamide or oral chlorambucil; IV cladribine, bortezomib).
BURKITT’S LYMPHOMA (Malignant Lymphoma of
https://t.me/medicina_free
Africa)
Thirty-eight cases of a sarcoma involving the jaws of African children
are described. This is a syndrome which has not previously been
fully recognised. It is by far the most common malignant tumour
of childhood seen at Mulago Hospital.
—Denis Parsons Burkitt, 1958
Burkitt’s lymphoma (BL) is a highly aggressive B cell NHL
characterized by the translocation and deregulation of the
c-MYC gene on chromosome 8 (8q24). BL comprises 30%
of pediatric lymphomas and <1% of adult non-Hodgkin
lymphomas. Endemic BL (eBL) corresponds to areas of high
malarial transmission in Africa.
It is common in South Africa and New Guinea. It is common
in children. It is common in males (4:1).
Epstien-Barr virus may be the aetiological agent. It is associ-
ated with infectious mononucleosis. It is common in malaria
endemic area.
The tumour is multifocal, rapidly growing and painless. Jaw
and abdomen are the commonest sites of involvement.
Microscopy: Primitive lymphoid cells with large clear histio-
cytes—starry night (starry sky) pattern.
It is common in jaw—either lower or upper. Maxilla is more
affected than the mandible; often painless; associated disfigurement, loosening and loss of teeth, halitosis, difficulty in
feeding and speech are common problems. The maxillary
tumour often spreads to involve the orbit, and presents as
proptosis, altered vision, and disfigurement.
Abdominal presentation is also common. Often involves the
spleen, liver, ovaries and kidneys (75%), lymph nodes. It
presents with masses, distention, pain, constipation, diarrhea,
difficulty breathing. Renal involvement often may be bilateral.
In females, ovaries are commonly affected.
CNS involvement (paraplegia, cranial nerve palsies) and
pleural effusion can occur. Orbit, bone marrow, peripheral
nodes can get affected occasionally.
Types a. Endemic (African)—commonly occurs in jaw. b.
Nonendemic (sporadic)—commonly occurs in abdomen.
c. Aggressive (immunodeficiency associated)—occurring
in HIV patients.
Investigations: FNAC and biopsy confirms the diagnosis.
Jaw X-ray shows osteolytic lesions. CT jaw, head and neck is
essential. Ultrasound abdomen is done to look for involvement
of kidneys. CT abdomen and thorax is helpful. Blood urea
and serum creatinine, LDH and serum uric acid estimation
is important. Bone marrow biopsy/aspiration are also done.
Differential diagnosis: Other jaw tumours, osteomyelitis, jaw
abscess, nephroblastoma, neuroblastoma, sarcomas.
Staging: (A)—Single Extra-abdominal tumor site. (AR)—
Completely (>90%) resected intra-abdominal tumor. (B)—
Multiple extra-abdominal tumor sites. (C)—Intra-abdominal
tumour with or without facial tumour. (D)—CNS and bonemarrow involvement.
St Jude’s staging: (1)—Local. (2)—Regional. (3)—Extensive.
(4)—Disseminated. This staging is important for therapy.
Treatment and prognosis
Initial hydration; allopurinol orally.
Chemotherapy: Vincristine, Actinomycin , Methotrexate iv/
intrathecal for CNS prophylaxis. Second line: Etoposide,
Cytarabine, Rituximab, Doxorubicin. Fluoxetine, cyclophosphamide, orthomelphalan also used.
Surgery is usually not indicated unless it is localised or in
case of involvement of ovaries. Debulking, decompression
(spine) surgeries are done only when needed.
Radiotherapy is often used.
Prognosis: Based on tumour burden (size); stage of the disease;
age >13 years; high serum LDH and uric acid levels. Recurrences
after 2 years are rare and exceptional after 3 years. Relapse rate
is 50%; mostly occur in first year; relapses often involve CNS;
relapse patients too achieve long-term remission of 4–10 years
commonly. Relapses in <3 months respond poorly.
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
most common among them.
Cutaneous T cell lymphoma can be indolent (commonly
mycosis fungoides); aggressive (Sezzary syndrome); provisional (granulomatous/panninculitis like T cell lymphoma).
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. Tumour cells in peripheral smear are also important
in deciding therapy and prognosis.
Multiple skin biopsies/peripheral smear/node biopsy/
immunohistochemistry/pheo or genotyping are important
investigations.
Prognosis depends on extent of skin involvement (more
than 10% body surface area carries poor prognosis)/nodal
spread/blood spread.
A
B
Figs. 1.458A and B: Cutaneous T cell lymphoma.
255
CHAPTER 1O General Surgery: Lymphatics
Courage is fear that has said its prayers.
Соседние файлы в папке @xirurgi_2025