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8 Haematology
Neutropenia: definitions and causes
https://t.me/med1917
Neutropenia refers to an absolute neutrophil count (
ANC
>1.0 ×
penia (
neutropenia (
rheumatological disorders, and haematologic conditions (eg
neutropenia (
pine, sulfasalazine, various antibiotics/
109/ L) in adults is most commonly attributable to benign ethnic
BEN
), drugs, infections (especially viral), nutritional deficiencies,
ANC
<0.5 ×
109/ L) is usually caused by drugs (eg carbimazole, cloza-
AED
ANC
) <1.5 ×
109/ L. Mild neutro-
MDS
). Severe isolated
S), and less commonly infections/ sepsis.
Neutropenia may also occur in the context of pancytopenia due to bone marrow
infiltration/ replacement (eg acute or chronic leukaemias), bone marrow failure (eg
aplastic anaemia, cytotoxic drugs, nutritional deficiencies, alcohol excess, viral
infection), excess destruction/ sequestration (eg
hypertension, etc.), or congenital conditions (eg Wiskott– Aldrich syndrome).
ISTH DIC
score
The
DIC
score was developed by the International Society of Thrombosis and
ISTH
Hemostasis (
• Platelet count, cells ×
• Fibrin degradation products (
• Prolonged
) as diagnostic criteria for overt
109/ L
D
- dimers)
PT
, seconds
• Fibrinogen level, g/ L
<5: not suggestive of overt
and manage clinically as appropriate.
5
: compatible with overt
≥
DIC
, may be non- overt
DIC
; treat for
DIC
, hypersplenism due to portal
DIC
:
100 0
>
–
100 1
50
<
50 2
No increase
Moderate increase
Severe increase
3 0
<
–
6 1
3
≥
6 2
1 0
≥
<
1 1
DIC
; repeat within next 1– 2 days
DIC
as appro pri ate an d rep eat s coring dail y.
0
2
3
351
Fig 8.
48
The appearance of disseminated
intravascular coagulation (
Courtesy of the Crookston Collection.
DIC
) on the sole.
8 Haematology
Acute lymphoblastic leukaemia (
ALL
CNS
OHCS
https://t.me/med1917
352
A malignancy of lymphoid cells, aecting B- or T- lymphocyte cell lineages, arresting
maturation and promoting uncontrolled proliferation of immature blast cells, with
marrow failure and tissue infiltration. Ionizing radiation (eg
nancy, and Down syndrome are important associations. It is the commonest cancer
of childhood, and represents about
common.
Classification Surface markers are used to classify
ALL, B
- cell
cell
ALL. WHO
molecular features. Cytogenetic features are characterized by chromosome analysis
and fluorescence in situ hybridization (
are used for mutation analysis and gene expression studies (
Signs and symptoms (fig
• Marrow failure: anaemia (Hb), infection (
• Infiltration: hepato- and splenomegaly, lymphadenopathy— superficial or medias-
tinal, orchidomegaly,
Common infections Especially chest, mouth, perianal, and skin. Bacterial septi-
CMV
caemia, zoster,
Te st s
FBC (WCC
, measles, candidiasis, Pneumocystis pneumonia (p
usually high)
blood or bone marrow to identify blast cells.
chromosomal abnormalities, eg
chromosome’.
CXR
and CT scan to look for mediastinal and abdominal lymphadenop-
athy. Lumbar puncture should be performed to look for
Treatment Educate and motivate patient to promote engagement with therapy.
• Support Blood/ platelet transfusion, IV fluids, allopurinol (prevents tumour lysis
syndrome). Insert a subcutaneous port system/ Hickman line for
• Infections These are dangerous, due to neutropenia caused by the disease and
treatment: give immediate
p
350
) and give prophylactic antivirals, antifungals, and antibiotics.
(
• Chemotherapy Prolonged chemotherapy is required for cure, eg
based clinical trials that can take up to
absence of the Philadelphia chromosome (Ph+
translocation of chromosomes
• Remission induction: eg vincristine, prednisolone,
• Consolidation: high- medium- dose therapy in ‘blocks’ over several weeks.
•
• Maintenance: prolonged chemotherapy, eg mercaptopurine (daily), metho-
BCR– ABL1
rubicin. A
bined with steroids and chemotherapy if it is a Ph+
prophylaxis: intrathecal (or high- dose IV) methotrexate ±
trexate (weekly), and vincristine + prednisolone (monthly) for
can occur in blood,
p
details:
• Matched related allogeneic marrow transplantations Once in 1st remission is
242
the best option in standard- risk younger adults.
Haematological remission Means no evidence of leukaemia in the blood, a normal
or recovering blood count, and <
Prognosis Cure rates for children are 70– 90%; for adults only 40% (higher when
imatinib/ rituximab,
chromosome, presentation with
used to detect minimal residual disease, undetectable by standard means. Prognosis
p
in relapsed Ph- negative
Personalized treatment Aim to tailor therapy to the exact gene defect, and
according to individual metabolism. New treatments for relapsed disease include
blinatumomab and inotuzumab.
Clinical trials are an option for patients with relapsed/ refractory disease. Biomarkers,
eg thiopurine methyltransferase, can predict toxicity from thiopurines.
)
X
- rays) during preg-
15
% of leukaemias in adults.
ALL
CNS
involvement is
into: precursor B- cell
ALL, T
classification depends on characterization of cytogenetic and
FISH
), while a variety of molecular techniques
8.53
) Due to:
CNS
involvement— eg cranial nerve palsies, meningism.
, PT, APTT,
BCR– ABL
IV
antibiotics. Start the neutropenic sepsis regimen
9
and 22):
WCC
), and bleeding (platelets).
fibrinogen,
DNA
fusion gene indicative of t(9:22) ‘Philadelphia
2
years. Depends on age and the presence/
tyrosine kinase inhibitor (eg imatinib, p
CNS
, or testis (examine these sites at follow- up). More
figs
8.49– 8.52
).
396
LFTS, U&E
. Immunophenotyping of
).
analysis is required to detect
CNS
invo lvement.
IV
access.
UKALL
regimen-
p
356
:
BCR– ABL
gene fusion due to
L
- asparaginase + dauno-
356
) should be com-
ALL.
CNS
irradiation.
2
yrs. Relapse
.
5
% blasts in a normal regenerating marrow.
356
, are used). Poor prognosis if: age ≥40, male, Philadelphia
CNS
signs, Hb,
ALL
is p oor (but impro vable by marrow transp lant).
CAR- T
WCC
>
100
×
109/ L, or B- cell
ALL. PCR
cells have shown excellent responses in
ALL
-
is
.
8 Haematology
Fig 8.
https://t.me/med1917
49
Blood film in
blasts with scanty cytoplasm.
Fig 8.
51
Blood film in
blast cells with greater morphological variation
and more abundant cytoplasm.
Fig 8.
53
Bilateral parotid infiltration in
(Enlarged salivary glands are also seen in
HIV
, bulimia, myxoedema, etc., p
mumps,
ALL, L1
subtype. Small
Courtesy of Prof. Christine Lawrence.
ALL, L2
subtype. Larger
Courtesy of Prof. Christine Lawrence.
ALL
586
.)
Fig 8.
50
Bone marrow in
Courtesy of Prof. Christine Lawrence.
Fig 8.
52
ALL L3
philic cytoplasm. (a, b) Blood films. (c) Lymph
node.
Courtesy of Prof. Tangün and Dr Köroğlu.
.
ALL, L1
subtype.
. Blasts with vacuolated baso-
353
8 Haematology
Acute myeloid leukaemia (
AML
BMT
https://t.me/med1917
354
Neoplastic proliferation of blast cells derived from marrow myeloid elements. It
progresses rapidly (
60
after ).
tients >
6
Incidence The commonest acute leukaemia of adults (1/ 10
age). The median age at presentation is
plication of chemotherapy, eg for lymphoma. Also associated with myelodysplastic
states (see
Down’s.
BOX
‘Myelodysplastic syndromes’), prior radiation, and syndromes, eg
Morphological classification Four types based on
cytogenetics, and molecular genetics:
1
AML
with recurrent genetic abnormalities.
2
AML
multilineage dysplasia (eg 2° to pre- existing myelodysplastic syndrome).
3
AML
, therapy related (in those previously treated with cytotoxic drugs).
4
AML
, other (not fitting above- listed).
Signs and symptoms • Marrow failure Anaemia, infection, or bleeding.
curs in acute promyelocytic leukaemia, a subtype of
of thromboplastin (
fig 8.54), skin involvement.
trophy (
Diagnosis
p
WCC
is often , but can be normal or even low. Blast cells may be
few in the peripheral blood, so diagnosis depends on bone marrow biopsy,
immunophenotyping, and molecular methods. On biopsy,
ALL
by Auer rods (figs
treatment recommendations and prognosis. ≥
ated with worse prognosis.
Complications • Predisposition to infection by both the disease and the treatment;
may be bacterial, fungal, or viral— prophylaxis is given for each during therapy. Be
alert to septicaemia (
can infect commonly (particularly the fungi Candida and Aspergillus). Be aware
AML
itself causes fever. • Chemotherapy causes plasma urate levels (from tu-
that
mour lysis)— so give allopurinol with chemotherapy, and keep well hydrated with
IV
fluids. • Leukostasis (p
high blast count. Manifestations include respiratory compromise, altered mental
status, visual changes, and headache.
leukapheresis, or radiotherapy.
Treatment • Supportive care As for
• Chemotherapy Very intensive, resulting in long periods of marrow suppression
with neutropenia + platelets . The main drugs used include daunorubicin and
cytarabine, with ~
occur in ~
• Bone marrow transplant (
5
20
% of patients with
lected from the marrow. Allogeneic transplants from
on international databases) are indicated in refractory or relapsing disease. The
idea is to destroy leukaemic cells and the immune system by, eg cyclophosphamide
+ total body irradiation, then repopulate the marrow with donor cells infused
Ciclosporin ± methotrexate are used to reduce the eect of the new marrow at-
tacking the patient’s body (graft vs host disease (
• Complications:
infections; relapse of leukaemia; infertility.
• Prognosis: lo wer r elap se rat es ~
of ~
10
%. Autologous
GVHD
selves) is used in intermediate- prognosis disease, although some studies suggest
better survival rates with intensive chemotherapy regimens.
haemopoietic recovery and less morbidity.
• Supportive care, or lower- dose chemotherapy for disease control, may be more
appropriate in elderly patients, where intensive therapies have poorer outcomes.
)
5
- year survival is now 55% for patients <60 and 17% for pa-
000
65– 70
years.
AML
WHO
350
). • Infiltration Hepatomegaly, splenomegaly, gum hyper-
CNS
involvement at presentation is rare.
8.55– 8.57
). Cytogenetic analysis (eg type of mutation) guides
p
350
): common organisms present oddly and rare organisms
350
) may occur if
3
cytogenetic abnormalities associ-
WCC
. This is associated with a very
/ yr; increases with
can be a long- term com-
histological classification,
AML,
where there is release
AML
is dierentiated from
DIC
IV fluids, rapid initiation of chemotherapy,
ALL
.
cycles given in 1- week blocks to get a remission (
AML
and enhance sensitivity to cytarabine).
) Pluripotent haematopoietic stem cells are col-
GVHD
HLA
)).
(may help explain the curative eect of
60
BMT
% long- term survivors, but significant mortality
(where stem cells are taken from the patient them-
RAS
mutations
- matched donors (held
BMT
); opportunistic
oc-
IV
.
8 Haematology
Fig 8.
https://t.me/med1917
54
Gum hypertrophy in
Courtesy of Prof. Christine Lawrence.
Fig 8.
56
AML
on the peripheral blood film.
with monoblasts and myeloblasts
Courtesy of Prof. Christine Lawrence.
AML.
Fig 8.
55
Auer rods (crystals of coalesced gran-
AML
ules) found in
Fig 8.
myeloblast cells.
Courtesy of Prof. Christine Lawrence.
57
Marrow in
AML:
multiple monoblasts.
Courtesy of Prof. Christine Lawrence.
355
Myelodysplastic syndromes (
These are a heterogeneous group of clonal disorders characterized by inadequate
and dysmorphic haematopoiesis that manifest as marrow failure with risk of lifethreatening infection and bleeding (median survival varies from
years according to disease type). Incidence increases with age. Mostly primary,
but can develop secondary to chemotherapy or radiotherapy.
to acute leukaemia.
Tests Pancytopenia (p
Marrow cellularity is usually increased due to ineective haematopoiesis. Ring
sideroblasts may also be seen in the marrow (
Dierential diagnosis
ACD
therapy), viral infections (
Tre at me nt
• Depends on age, performance status, and prognostic scoring systems.
• Multiple transfusions of red cells or platelets as needed. Chelation therapy may be
needed for iron overload.
• Erythropoietin may lower transfusion requirement. G-
• Allogeneic stem cell transplantation is one option (curative but often inappropriate
owing to age- related comorbidities— most are >
• Low- intensity treatments that are not curative but may improve quality of life
in symptomatic disease include thalidomide analogues (eg lenalidomide) or
hypomethylating agents (eg azacitidine and decitabine).
MDS
, myelodysplasia)
6
months to 6
30
348
), with reticulocyte count,
fig
8.25
, p
327
% transform
).
, marrow infiltration, drug toxicity (eg alcohol, chemo-
HIV
), vitamin deficiency (
B
/ folate deficiency).
12
CSF
if recurren t infe ctions.
70
yrs old).
MCV
.
8 Haematology
Chronic myeloid leukaemia (
CML
https://t.me/med1917
356
CML
is characterized by an uncontrolled clonal proliferation of myeloid cells (fig
It accounts for
features in common with these diseases, eg splenomegaly. It occurs most often
between
associated with prior radiation exposure.
15
% of leukaemias. It is a myeloproliferative disorder (p
40– 60
yrs, with a slight male predominance, and is rare in childhood. Risk
Philadelphia chromosome (Ph.) Present in >80% of those with
chromosome comprising reciprocal translocation between the long arm of chromo-
9
and the long arm of chromosome 22— t(9;22)— forming a fusion gene
some
ABL
on chromosome 22, which has tyrosine kinase activity. Those without Ph have a
worse prognosis. Some patients have a masked translocation— cytogenetics do not
show the Ph, but the rearrangement is detectable by molecular techniques.
Symptoms Mostly chronic and insidious: weight, tiredness, fever, sweats. There
may be features of gout (due to purine breakdown), bleeding (platelet dysfunction),
and abdominal discomfort (splenic enlargement). ~
Signs Spl enome galy ( >75%)— often massive. Hepatomegaly, anaemia, bruising (fig
Te st s
WBC
(often >10 ×
monocytes, basophils, eosinophils. Hb or , platelets variable. Urate,
marrow hypercellular. Cytogenetic analysis of blood or bone marrow for Ph.
Natural history 5- year survival is 90%. There are three phases: chronic, lasting
months or years of few, if any, symptoms.
blast cells in peripheral blood or bone marrow as per
toms and spleen size.
Treatment See
death.
Fig 8.
58
CML
at dierent stages of dierentiation.
Treating
CML
: numerous granulocytic cells
Courtesy of Prof. Christine Lawrence.
CML
is the first example of a cancer where knowledge of the genotype has led to
a specifically targeted drug— imatinib, a
has transformed therapy over the last
cramps, oedema, rash, headache, arthralgia. May cause myelosuppression.
More potent
and ponatinib. Dasatinib and nilotinib allow more patients to achieve deeper,
2
nd- generation
more rapid responses associated with improved outcomes, but are only indicated in chronic- or accelerated- phase patients who are intolerant or resistant to
imatinib according to
Those with lymphoblastic transformation may benefit from treatment as for
ALL
. Treatment of myeloblastic transformation with chemotherapy rarely achieves
lasting remission, and allogeneic transplantation oers the best hope.
Stem cell transplantation: allogeneic transplantation from an
sibling or unrelated donor oers the only cure, but carries significant morbidity
and mortality. Guidelines suggest that this approach should be only rarely used
1
st line in young patients (where mortality rates are lower). Other patients should
be oered a
whether to continue, to oer combination therapy or stem cell transplantation.
BCR– ABL
)
8.58
362
) having
CML
. It is a hybrid
BCR
30
% are detected by chance.
8.59
109/ L) with whole spectrum of myeloid cells, ie neutrophils,
B
. Bone
12
• Accelerated phase, defined as 10– 19%
WHO
with increasing symp-
• Blast transformation, with features of acute leukaemia ±
BOX
.
Fig 8.
59
BCR– ABL
10
yrs. Side eects are usually mild: nausea,
BCR– ABL
inhibitors: dasatinib, nilotinib, bosutinib,
NICE
. Hydroxycarbamide is also used.
Hepatosplenomegaly in
tyrosine kinase inhibitor. This
CML
HLA
- matched
.
inhibitor. Patients are then reviewed annually to decide
).
/
).
8 Haematology
Chronic lymphocytic leukaemia (
CLL
https://t.me/med1917
CLL
is the commonest leukaemia (>25%; incidence: ~5/
median age at diagnosis is
a malignant clone of functionally incompetent
deletions (eg del
Table 8.
Rai stage:
17p13
8
Staging and survival in
0
I
II
III
IV
Symptoms Often none, presenting as a surprise finding on a routine
65
years. The hallmark is progressive accumulation of
) influence risk (table
Lymphocytosis alone
Lymphocytosis + lymphadenopathy
Lymphocytosis + spleno- or hepatomegaly
Lymphocytosis + anaemia (Hb <
Lymphocytosis + platelets <
CLL
)
100 000
/ yr). : ≈ 2:1. The
B
cells. Mutations, trisomies, and
8.8
).
Median survival >
110
g/ L)
100
×
109/ L
8
yrs
5
yrs
2
yrs
1
yr
FBC
13
yrs
. Patients
may be anaemic or infection- prone, or have weight, sweats, anorexia if severe.
Signs Enlarged, rubbery, non- tender nodes (fig
Tests Lymphocytes— may be marked (fig
p
334
), marrow infiltration: Hb, neutrophils, platelets.
(
8.60
). Splenomegaly, hepatomegaly.
8.61
). Later: autoimmune haemolysis
Complications 1 Autoimmune haemolysis. 2 Bacterial infections due to
IGG
hypogammaglobulinaemia (=
T cell immunosuppression with increased susceptibility to viral (eg herpes zoster)
and fungal infections.
of transformation to more aggressive disease (consider if rapidly enlarging nodes,
rapid increase in
4
Marrow failure. 5 Risk of secondary malignancies. 6 Risk
LDH, B
symptoms).
), especially encapsulated organisms. 3 Severe
Treatment Depends on age/ fitness and the presence of certain mutations.
Many patients can be managed on a ‘watch and wait’ basis safely for several
years. Consider treatment if symptomatic. Options include
cyclophosphamide + rituximab), ibrutinib (Bruton tyrosine kinase inhibitor;
AF
myelosuppression,
mour lysis syndrome, myelosuppression) + obinutuzumab, and
rituximab). Steroids and
after treatment with fludarabine).
splenomegaly.
Supportive care Transfusions, IV human immunoglobulin if recurrent infec-
tients.
, and bleeding) monotherapy, venetoclax (
IV
immunoglobulin help autoimmune haemolysis (seen
Radiotherapy helps lymphadenopathy and
Stem- cell transplantation may have a role in carefully selected pa-
FCR
(fludarabine +
BCL- 2
inhibitor; SE: tu-
BR (
bendamustine +
SE
tion ± antimicrobial prophylaxis
Natural history ⅓ never progress (or even regress), ⅓ progress slowly, and ⅓ pro-
gress actively.
CD23
and 2- microglobulin correlate with bulk of disease and rates
of progression. Death is often due to infection or transformation to aggressive
lymphoma (Richter’s syndrome).
357
:
Fig 8.
60
Bilateral cervical lymphadenopathy
CLL
.
in
Fig 8.
61
CLL
: many lymphocytes and a ‘smear’
cell: a fragile cell damaged in preparation.
Courtesy of Prof. Christine Lawrence.
8 Haematology
Hodgkin’s lymphoma (HL)
I
II
III
IV
https://t.me/med1917
358
Lymphomas are disorders caused by malignant proliferations of lymphocytes. These
accumulate in the lymph nodes causing lymphadenopathy, but may also be found
in peripheral blood or infiltrate organs. Lymphomas are histologically divided into
Hodgkin’s and non- Hodgkin’s types. In Hodgkin’s lymphoma,
with mirror- image nuclei are found, called Reed– Sternberg cells (
Incidence Two peaks: young a dults (HL is th e commonest maligna ncy in 15– 24yr- olds)
and elderly. :
sibling;
EBV
≈
2:1
(p
401
);
SLE
Symptoms Often presents with enlarged, non- tender, ‘rubbery’ superficial lymph
60– 70
nodes (
they can become matted.
in
node pain. Mediastinal lymph node involvement can cause mass eect, eg bronchial
or
% cervical, fig
6
months), night sweats, pruritus, and lethargy. There may be alcohol- induced lymph
SVC
obs truction (p
Signs Lymphadenopathy. Also, cachexia, anaemia, spleno- or hepatomegaly.
Te st s Tissue diagn osis Lymp h node excision biopsy i f possi ble (su praclavicular > cer-
vical/ axillary > inguinal; send for pathology, immunohistochemisty/ flow cytometry).
Image- guided needle biopsy, laparotomy, or mediastinoscopy may be needed.
FBC
, film,
ESR, LFT, LDH
released during cell turnover.
Staging (Ann Arbor system.) Influences treatment and prognosis. Done by imaging
B
± marrow b iopsy i f
Confined to single lymph node region.
Involvement of two or more nodal areas on the same side of the diaphragm.
Involvement of nodes on both sides of the diaphragm.
Spread beyond the lymph nodes, eg liver or bone marrow.
Each stage i s either ‘
B
symptoms: weight loss >10% in last 6 months, unexplained fever >38°C, or night
of
symptom s, or s tage
sweats (needing change of clothes). ‘
extension does not advance the stage, but is indicated by subscripted
Chemoradiotherapy Radiotherapy + short courses of chemotherapy for stages
I- A
and
II- A
(eg with ≤3 areas involved). Longer courses of chemotherapy for
3
areas involved through to
with >
Vinblastine, Dacarbazine cures ~80% of patients. More intensive regimens are
used if poor prognosis or advanced disease.
therapy followed by autologous stem cell transplantation. Brentuximab vedotin +
doxorubicin, vinblastine, and dacarbazine (
chemotherapy.
7
Complications of treatment See pp
of second malignancies (related to dose and field, with long latency and risk over
time), solid tumours (especially lung and breast, also melanoma, sarcoma, stomach,
and thyroid cancers), ischaemic heart disease, valvular heart or pericardial disease, hypothyroidism, and lung fibrosis due to the radiation field. Chemotherapy
SE
include myelosuppression, nausea, alopecia, infection.
lymphoma, and infertility may be due to both chemo- and radiotherapy— see
5
- year survival Depends on stage and grade (table
predominant disease; <
risks in terms of overall survival between minimizing the risk of a recurrence and
also preventing treatment- related complications such as secondary malignancy and
cardiovascular disease.
Emergency presentations Infection;
in the head, dyspnoea, blackouts, facial oedema (seek expert help; see
8
Thomas Hodgkin (
disease Hodgkin’s name.
9
Eg
BEACOPP
In
IIB, III
1798– 1866
(bleomycin/ etoposide/ doxorubicin/ cyclophosphamide/ vincristine/ procarbazine/ prednisone).
, or IV,
BEACOPP
gi ve s b e tt er in it ia l co nt ro l, bu t 7yr event- free survival is similar: 78% vs 71%.
8
characteristic cells
figs
8.62– 8.64
).
. Incidence in industrialized countries. Risk factors An aected
; post- transplantation.
8.65
; also axillary or inguinal). Node size may fluctuate, and
25
% have constitutional upset, eg fever, weight loss (> 10%
524
), or direct extension, eg causing pleural eusions.
2
+
, urate, Ca
A
.
ESR
or Hb indicate a worse prognosis.
Imaging
CXR, CT/ PET
of thorax , abdo , and pelvis.
III– IV
dis ease.
’— no systemic symptoms other than pruritus; or
B
’ indicates worse disease. Localized extranodal
IV- B. ‘ABVD
’: Adriamycin® (doxorubicin), Bleomycin,
9
In relapsed disease: high- dose chemo-
BV
+
AVD
) is a promising new combination
518– 21
. Radiotherapy may risk
AML
(p
354
8.9
40
% with
IV- B
lymphocyte- depleted. There are competing
SVC
obstruction—
); rediscovered by Samuel Wilks (
): >95% in
JVP
, sensation of fullness
1824– 1911
) who magnanimously gave the
Bloods
LDH
as it is
‘B’
— presence
‘E’
, eg
I- A
.
E
II- A
), non- Hodgkin’s
p
523
I- A
lymphocyte-
p
524
).
.
8 Haematology
Table 8.
HIV
https://t.me/med1917
9
HL subtypes
Classification (% of cases)
Nodular sclerosing (70%)
Mixed cellularity* (
Lymphocyte rich (
Lymphocyte depleted* (<
NB
: nodular lymphocyte predominant Hodgkin’s is recognized as a separate entity,
behaving as an indolent
Higher incidence and worse prognosis if
*
Fig 8.
62
A Reed– Sternberg cell with two nu-
clei, characteristic of Hodgkin’s lymphoma.
Fig 8.
64
Mononuclear Reed– Sternberg cell in
a lymph node.
20– 25
%)
5
%)
1
%)
B
- cell lymphoma.
Courtesy of Prof. Christine Lawrence.
© Prof. Tangün and Dr Köroğlu.
Prognosis
Good
Good
Good
Poor
+ ve.
Fig 8.
63
Another Reed– Sternberg cell.
Courtesy of the Crookston Collection.
Fig 8.
65
Cervical lymphadenopathy in Hodgkin’s
disease.
359
Quality of life, lymphoma, and the role of expressive writing
Being treated for Hodgkin’s lymphoma is arduous. Our job is often to give encouragement— the more this is personalized for our individual patient the better.
One method is to encourage our patients to write about their experiences. In
one study this gave clear- cut benefits in lymphoma patients. Participants report
positive responses to writing, and half said that writing changed their thoughts
about their illness in a positive way (this increased on subsequent follow- up).
Textual analysis identifies themes related to experiences of positive change, transformation, and self- armation through reflection. These techniques are akin to
those used in post- traumatic stress— and remind us that some of our treatments
are as destabilizing to our patients as any shipwreck or earthquake. ‘I can whine, I
can complain, I can moan, and bitch, about all of the above, but I won’t. . . The true
feat isn’t escaping death, rather, learning how to live.’
Sometimes narrating lymphoma experiences reveals bitterness, loss of control,
and a feeling that life has been rendered void. Here our role is to receive these
negatives and to try to keep the channels of communication open, as dialogue
is the only validated means of filling these voids. The need to enhance support
networks and bolster social ties may trump all our pharmacological imperatives.
8 Haematology
Non- Hodgkin’s lymphoma
MALT
R- CHOP
https://t.me/med1917
360
This includes all lymphomas without Reed– Sternberg cells (p
group. They can be classified as
derived from
B
- cell lines; diuse large B- cell lymphoma (
all centre on nodes (extranodal tissues generating lymphoma include mucosaassociated lymphoid tissue, eg gastric
doubled since
1970
(to 2:10
Causes Immunodeficiency— drugs; prior radiotherapy or chemotherapy; viruses
HIV
or bacteria—
EBV
is also associated with endemic Burkitt’s lymphoma,
HTLV- 1
etc.);
(usually high- grade lymphoma from
(adult
T-
zone lymphoma); toxins; autoimmune condition; congenital.
Signs and symptoms • Superficial lymphadenopathy (75% at presentation).
• Extranodal disease (50%). Gut (Commonest.) 1 Gastric
and may regress with its eradication (
with systemic features (see below).
and metastasizes late.
B
large- cell
cation.
disease,
lymphoma)— presents with diarrhoea, vomiting, abdominal pain, and weight.
Poor prognosis.
fungoides (accounts for ~
causes sore throat/ obstructed breathing.
• Systemic features: low- grade- NHL— none/ mild symptoms, lymphadenopathy; high-
grade- NHL— weight loss, night sweats, fevers.
• Pancyto penia f rom mar row involvem ent— anaemia, infection, bleeding (platelets).
Te st s Blood
and node biopsy
grade).
cytology of any eusion; LP for
lymphomas— high- grade and not responding well to H. pylori eradi-
3
Small- bowel lymphomas, eg
p
364
), or
Skin (2nd commonest— see fig 8.66.) Eg clonal T cells in mycosis
FBC, U&E, LFT
for classification (complex, based on the
Staging Ann Arbor system (p
Diagnosis/ management This is multidisciplinary, synthesizing details from clinical
evaluation, histology, immunology, molecular genetics, and imaging. Generally:
• Low- grade lymphomas are indolent, often incurable, and widely disseminated.
Include: follicular lymphoma (
lymphoma/
MALT
, lymphocytic lymphoma (closely related to
similarly), lymphoplasmacytoid lymphoma (produces
macroglobulinaemia,
• High- grade lymphomas are more aggressive, but often curable. There is often
rapidly enlarging lymphadenopathy with systemic symptoms. Include: Burkitt’s
lymphoma (childhood disease with characteristic jaw lymphadenopathy;
8.69
), lymphoblastic lymphomas (like
common subtype
NHL
Treatment Huge range of options, depending on disease subtype. Low grade If
symptomless, none may be needed (see
calized disease. Chlorambucil is used in diuse disease. Remission may be maintained by using interferon alfa or rituximab (see later in paragraph). Bendamustine
is eective both with rituximab and as a monotherapy in rituximab- refractory pa-
High grade (eg large B- cell lymphoma,
tients.
Cyclophosphamide, Hydroxydaunorubicin, vincristine (Oncovin®) and Prednisolone.
Granulocyte colony- stimulating factors (
lenograstim (at low doses it may be cost- eective).
Survival Histology is important. Prognosis is worse if, at presentation: • Age
>
• Disseminated disease. Typical 5yr survival for treated patients: ~30% for high-
grade and >
50
% for low- grade lymphomas, but the picture is very variable.
B
- cell or T- and
000
). The median age at diagnosis is 67 years.
cell leukaemia/ lymphoma) p
2
Non- malt gastric lymphomas (60%) are usually diuse
EATCL (
MALT
enteropathy/ coeliac- associated intra- epithelial T- cell
50
%— p
588
NK-
cell derived. However, most are
DLBCL
) is commonest. Not
MALT
, later in this topic). Incidence has
EBV
transformed cells, p
DLBCL, NK– T-
401
; Helicobacter pylori (marginal
p
248
). Symptoms: as for gastric Ca (p
cell lymphoma,
is caused by H. pylori,
usually involves the antrum, is multifocal,
IPSID
(immunoproliferative small intestine
). Oropharynx Waldeyer’s ring lymphoma
Other possible sites Bone,
358
.
LDH
≈ worse prognosis, reflecting cell turnover. Marrow
358
)—
PET- CT
cytology if
CNS
nd most common
8.67
.
ALL
), diuse large B- cell lymphoma (most
p
364
CSF
2
). See fig
WHO
system of high or low
of chest, abdomen, pelvis. Send
signs.
NHL
— 20%), marginal zone
CLL
and treated
IgM
= Waldenström’s
— 25%).
BOX
). Radiotherapy may be curative in lo-
DLBCL
), ‘
’ regimen: Rituximab,
G- CSF
S) help neutropenia— eg filgrastim or
)— a diverse
401
610
CNS
, and lung.
figs
8.68
;
),
,
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