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Lecture 10
Developed by - Dr.Abdulrazzaq Othman Alagbare – MD-MCP- Lecturer of hematology &
Immunohematology 1
Hematologic Malignancies
Topic
Pediatric
non-Hodgkin
lymphoma
nhl
2
Pediatric NON -Hodgkin
lymphoma-NHL
3
The non-Hodgkin’s lymphomas are a heterogeneous group of
neoplastic disorders of lymphoid tissue,
Each group has different
 clinical,
 morphological,
 immunological,
 and genetic characteristics.
introduction
4
• NHL is categorized into immature and mature B or T-cell NHL.
• NHL is a systemic illness
• NHL is the fifth most common diagnosis of pediatric cancer in
• 6% childhood cancer , 60% of childhood lymphomas
• children under the age of 15 years
introduction
5
Non-Hodgkin lymphoma (NHL) – INFO
 develops in the lymph nodes or in lymphatic tissue
 in one or many sites in the body (nodal, extranodal)
 Lymphoid tissue : found in organs such as the stomach,
intestines BM. CNS or skin.
6
Non-Hodgkin lymphoma (NHL) – INFO
 Share in some characteristics
 Caused by DNA injury of a lymphocyte
 It is acquired
 Affected 50-60yrs old
7
and children
8
lymphoma can start in
lymph nodes
spleen
bone marrow
thymus
adenoids and tonsils
digestive tract lymphnodes
9
B cells NHL
lymphoma
10
Follicular lymphoma -FL
11
Indolent lymphoma: slow growing and spreading
 The most common form of NHL is the follicular lymphoma
 Why called FL? abnormal B lymphocytes (cancerous cells) grow in lymph
nodes in clusters to form nodules or follicles in the tissues they invade
(histological feature)
 It is a low grade lymphoma.
Follicular lymphoma -FL
12
Consist 21% of B cells lymphoma
Specific B cells are uncontrolled division
Affected people over 50 yrs
Rare in children
Follicular lymphoma -FL
13
Indolent lymphoma: slow growing and spreading
Not need therapy- wait &watch closely
Difficult to cure
Can transformed to
Diffuse large B cell lymphoma DLBCL
follicular lymphoma
14
Localized disease
Single lymph node
Above the diaphragm
Two or more
lymph nodes
Above the
diaphragm
Two or more
lymph nodes
Above or lower
the diaphragm
Wide spread
multiple organs
Above or lower the
diaphragm
Murphy staging system
15
include:
A symptoms
•Swollen glands (the lymph nodes)
•Sites: in the neck, armpit, abdomen, or groin,
•Pain: painless;
•B symptoms
•Deeper located : associated with pain
•Loss of appetite or weight
•Fever of unknown reason
•Tiredness and ongoing fatigue
•Excessive sweating
The classic symptoms of follicular lymphoma
16
include:
 History of the patient
 Physical examination: have painless enlargement of
superficial lymph nodes of small to medium size
Diagnosis of FL
17
18
include:
Blood tests
 CBC -This is done to rule out leukaemia
 a bone marrow biopsy –(stage VI)
 Blood chemistry : Serum LDH and β2-microglobulin are useful prognostic
markers in malignant lymphomas.
Diagnosis
Histological test
Gives Accurate diagnosis
 a lymph node biopsy (in which all or part of the lymph node is
removed and examined) is needed to confirm the diagnosis.
a tumor markers
19
 PET/CT scan  PET-CT is more sensitive and specific
(stage and localized of FL)
 x-rays,
 CT scans,
Radiological tests -Diagnosis
 immunophenotyping:Tumor cells are positive for the B-cell markers
CD10, CD19, CD22, and CD20, but always negative for CD5
 Cytogenetic analysis: t (14;18) which turns on the bcl-2 oncogene
leading to uncontrolled proliferation of B-cells
Immunophenotyping –Cytogenetic analysis
positron emission tomography -PET
20
Diffuse large B cell
lymphoma - DLBCL
21
Aggressive lymphoma: fast growing and spreading
The most common is the diffuse large B cell lymphoma (DLBCL)
high-grade (fast growing) non-Hodgkin lymphoma.
Consist 31% of B cells lymphoma
DLBCL accounts for about 8 to 10% of all NHL in childhood.
Diffuse large B cell lymphoma - DLBCL
22
It is called diffuse large B-cell lymphoma because:
 it develops from abnormal B cells
 the abnormal cells are larger than normal, healthy B cells
 the abnormal cells are spread out (diffuse) rather than grouped together
when they’re examined under a microscope
Diffuse large B cell lymphoma - DLBCL
23
B symptoms are observed in approximately
30% of patients
Need immediate therapy
Response will to treatment
Diffuse large B cell lymphoma - DLBCL
24
Types of
Diffuse large B cell
lymphoma - DLBCL
25
Primary mediastinal large B-cell lymphoma (PMLBL)
 accounts for 2-4% of all non-Hodgkin
lymphomas.
 Age: children and adults (25-40yrs of age)
 Gender :It is twice as common in women as
in men
 Site: Develops in the mediastinum (in the
chest)
 Symptoms: include breathlessness, persistent cough, difficulty swallowing
(dysphagia), swelling of the neck and face, dizziness and headaches.
 Treatment :involves chemotherapy, often followed by radiotherapy
26
T-cell/histiocyte-rich large B-cell lymphoma
 accounts 10% of people with DLBCL develop this type.
 Cells: three types of cells present- T-cells, histiocytes and large B-cells
(histopathology finding)
 Site: Develops in liver,spleen, abdomen
 Symptoms: include Symptoms include swollen lymph nodes, fever and
swelling of the liver , spleen and abdominal swelling
 Treatment :involves chemotherapy, often followed by radiotherapy
27
Anaplastic Large Cell
Lymphoma (ALCL)
T-cells lymphoma
28
Anaplastic
 A term used to describe cancer cells
that divide rapidly and have little or
no resemblance to normal cells.
 Anaplasia is a cellular condition
characterized by inadequate
differentiation
Anaplastic Large Cell Lymphoma (ALCL)
Definition
 ALCL is a cancer of mature T-cell
 It is a form of NHL.
 Consist about 10% of NHL in children
Sites
 May be localized
 May be advanced (widespread in chest, skin. lungs, abdomen or bones)
With fever,weight loss ,(B-symptoms)
Markers
 All has CD30 positive
 Some ALK-GENE positive (>90%)
 Some are ALK-GENE
 chromosomal translocation t(2;5) (85% of cases)
Anaplastic Lymphoma Kinase gene
30
Anaplastic large cell lymphoma (ALCL)
1-Primary systemic ALCL, ALK-positive,
is a fast-growing (aggressive) cancer, involve many organs.
most common in children and young adults.
This type of cancer usually responds well to chemotherapy treatments.
Anaplastic Lymphoma Kinase gene
Types
2-Primary systemic ALCL, ALK-negative,
is an aggressive cancer that
primarily affects older adults.
Hard treatment, Has relapse post therapy with Worse prognosis
31
Anaplastic large cell lymphoma
(ALCL)
Types
3-Primary cutaneous ALCL
causes skin changes, like bumps
or a rash.
In most cases (90% of the time),
it doesn’t spread beyond the
skin.
32
Figure 1 Skin rashes (A) before treatment and (B) after chemotherapy]
33
Anaplastic large cell lymphoma
(ALCL)
Types
4-Breast implant-associated ALCL
(BIA-ALCL)
forms around breast implants.
BIA-ALCL is usually diagnosed
about 10 years following a breast
augmentation or breast
reconstruction procedure.
34
Lymphoblastic Lymphoma (LL)
35
Lymphoblastic Lymphoma (LL)
Cells affected
 immature
 T- lymphocyte  T-LL
 B-lymphocyte B-LL
 It is one of the three most common types of NHL In children
 Present in similar way to one another and similar to ALL
 Age:0-15yrs –more common between 10-14 yrs.
Types of Lymphoblastic Lymphoma (LL)
36
T-Lymphoblastic Lymphoma (TLL)
 TLL arise from precursor T lymphoblasts at different stages of
differentiation
 Consist 30% of all NHL of children
 It is aggressive
 Good prognosis 85% cured
37
 consist about 80% of childhood LL
sites:
 thymus present as a mediastinal mass
(mass behind the breast bone but in
front of the windpipe, with breathing
shortness)
Precursor T-cell lymphoblastic Lymphoma
38
39
Precursor B-cell lymphoblastic Lymphoma
consist about 20% of childhood LL
sites: Begins in lymph nodes outside the mediastinum (Neck, tonsils, or
skin tumors)
40
Symptoms of Lymphoblastic Lymphoma -LL
the first symptoms notice a lump or several lumps, don't go away
 sites of lumps; head, neck, chest, armpit or groin (are swollen lymph nodes)
 advance stages: can spread to the lungs, (mediastinal mass) , liver, bones
lumps
41
the common symptoms of lymphoblastic lymphoma include
painless swelling of lymph nodes
easy bruising, persistence infections
advance stages: can spread to the lungs, (mediastinal mass)
shortness of breath, Cough, fatigue, prurittus
42
Target for best treatmeant
 Early stage (stage 1 and 2)
 Advanced stage (stage 3 and 4)
43
 Lymph node biopsy
 B.M biopsy (Leukemia or Lymphoma) 25% or more blasts in
the bone marrow
 CBC, ESR, LFT
 Chest X-ray, PET/CT positron emission tomography -PET
44
Differences between
Acute Lymphoblastic Leukemia vs Lymphoblastic Lymphoma
 Are the same clinically and microscopicaaly
45
46

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Pediatric Non Hodgkin lymphoma NEW.pdf

  • 1. Lecture 10 Developed by - Dr.Abdulrazzaq Othman Alagbare – MD-MCP- Lecturer of hematology & Immunohematology 1 Hematologic Malignancies
  • 4. The non-Hodgkin’s lymphomas are a heterogeneous group of neoplastic disorders of lymphoid tissue, Each group has different  clinical,  morphological,  immunological,  and genetic characteristics. introduction 4
  • 5. • NHL is categorized into immature and mature B or T-cell NHL. • NHL is a systemic illness • NHL is the fifth most common diagnosis of pediatric cancer in • 6% childhood cancer , 60% of childhood lymphomas • children under the age of 15 years introduction 5
  • 6. Non-Hodgkin lymphoma (NHL) – INFO  develops in the lymph nodes or in lymphatic tissue  in one or many sites in the body (nodal, extranodal)  Lymphoid tissue : found in organs such as the stomach, intestines BM. CNS or skin. 6
  • 7. Non-Hodgkin lymphoma (NHL) – INFO  Share in some characteristics  Caused by DNA injury of a lymphocyte  It is acquired  Affected 50-60yrs old 7 and children
  • 8. 8
  • 9. lymphoma can start in lymph nodes spleen bone marrow thymus adenoids and tonsils digestive tract lymphnodes 9
  • 12. Indolent lymphoma: slow growing and spreading  The most common form of NHL is the follicular lymphoma  Why called FL? abnormal B lymphocytes (cancerous cells) grow in lymph nodes in clusters to form nodules or follicles in the tissues they invade (histological feature)  It is a low grade lymphoma. Follicular lymphoma -FL 12
  • 13. Consist 21% of B cells lymphoma Specific B cells are uncontrolled division Affected people over 50 yrs Rare in children Follicular lymphoma -FL 13
  • 14. Indolent lymphoma: slow growing and spreading Not need therapy- wait &watch closely Difficult to cure Can transformed to Diffuse large B cell lymphoma DLBCL follicular lymphoma 14
  • 15. Localized disease Single lymph node Above the diaphragm Two or more lymph nodes Above the diaphragm Two or more lymph nodes Above or lower the diaphragm Wide spread multiple organs Above or lower the diaphragm Murphy staging system 15
  • 16. include: A symptoms •Swollen glands (the lymph nodes) •Sites: in the neck, armpit, abdomen, or groin, •Pain: painless; •B symptoms •Deeper located : associated with pain •Loss of appetite or weight •Fever of unknown reason •Tiredness and ongoing fatigue •Excessive sweating The classic symptoms of follicular lymphoma 16
  • 17. include:  History of the patient  Physical examination: have painless enlargement of superficial lymph nodes of small to medium size Diagnosis of FL 17
  • 18. 18
  • 19. include: Blood tests  CBC -This is done to rule out leukaemia  a bone marrow biopsy –(stage VI)  Blood chemistry : Serum LDH and β2-microglobulin are useful prognostic markers in malignant lymphomas. Diagnosis Histological test Gives Accurate diagnosis  a lymph node biopsy (in which all or part of the lymph node is removed and examined) is needed to confirm the diagnosis. a tumor markers 19
  • 20.  PET/CT scan  PET-CT is more sensitive and specific (stage and localized of FL)  x-rays,  CT scans, Radiological tests -Diagnosis  immunophenotyping:Tumor cells are positive for the B-cell markers CD10, CD19, CD22, and CD20, but always negative for CD5  Cytogenetic analysis: t (14;18) which turns on the bcl-2 oncogene leading to uncontrolled proliferation of B-cells Immunophenotyping –Cytogenetic analysis positron emission tomography -PET 20
  • 21. Diffuse large B cell lymphoma - DLBCL 21
  • 22. Aggressive lymphoma: fast growing and spreading The most common is the diffuse large B cell lymphoma (DLBCL) high-grade (fast growing) non-Hodgkin lymphoma. Consist 31% of B cells lymphoma DLBCL accounts for about 8 to 10% of all NHL in childhood. Diffuse large B cell lymphoma - DLBCL 22
  • 23. It is called diffuse large B-cell lymphoma because:  it develops from abnormal B cells  the abnormal cells are larger than normal, healthy B cells  the abnormal cells are spread out (diffuse) rather than grouped together when they’re examined under a microscope Diffuse large B cell lymphoma - DLBCL 23
  • 24. B symptoms are observed in approximately 30% of patients Need immediate therapy Response will to treatment Diffuse large B cell lymphoma - DLBCL 24
  • 25. Types of Diffuse large B cell lymphoma - DLBCL 25
  • 26. Primary mediastinal large B-cell lymphoma (PMLBL)  accounts for 2-4% of all non-Hodgkin lymphomas.  Age: children and adults (25-40yrs of age)  Gender :It is twice as common in women as in men  Site: Develops in the mediastinum (in the chest)  Symptoms: include breathlessness, persistent cough, difficulty swallowing (dysphagia), swelling of the neck and face, dizziness and headaches.  Treatment :involves chemotherapy, often followed by radiotherapy 26
  • 27. T-cell/histiocyte-rich large B-cell lymphoma  accounts 10% of people with DLBCL develop this type.  Cells: three types of cells present- T-cells, histiocytes and large B-cells (histopathology finding)  Site: Develops in liver,spleen, abdomen  Symptoms: include Symptoms include swollen lymph nodes, fever and swelling of the liver , spleen and abdominal swelling  Treatment :involves chemotherapy, often followed by radiotherapy 27
  • 28. Anaplastic Large Cell Lymphoma (ALCL) T-cells lymphoma 28
  • 29. Anaplastic  A term used to describe cancer cells that divide rapidly and have little or no resemblance to normal cells.  Anaplasia is a cellular condition characterized by inadequate differentiation
  • 30. Anaplastic Large Cell Lymphoma (ALCL) Definition  ALCL is a cancer of mature T-cell  It is a form of NHL.  Consist about 10% of NHL in children Sites  May be localized  May be advanced (widespread in chest, skin. lungs, abdomen or bones) With fever,weight loss ,(B-symptoms) Markers  All has CD30 positive  Some ALK-GENE positive (>90%)  Some are ALK-GENE  chromosomal translocation t(2;5) (85% of cases) Anaplastic Lymphoma Kinase gene 30
  • 31. Anaplastic large cell lymphoma (ALCL) 1-Primary systemic ALCL, ALK-positive, is a fast-growing (aggressive) cancer, involve many organs. most common in children and young adults. This type of cancer usually responds well to chemotherapy treatments. Anaplastic Lymphoma Kinase gene Types 2-Primary systemic ALCL, ALK-negative, is an aggressive cancer that primarily affects older adults. Hard treatment, Has relapse post therapy with Worse prognosis 31
  • 32. Anaplastic large cell lymphoma (ALCL) Types 3-Primary cutaneous ALCL causes skin changes, like bumps or a rash. In most cases (90% of the time), it doesn’t spread beyond the skin. 32
  • 33. Figure 1 Skin rashes (A) before treatment and (B) after chemotherapy] 33
  • 34. Anaplastic large cell lymphoma (ALCL) Types 4-Breast implant-associated ALCL (BIA-ALCL) forms around breast implants. BIA-ALCL is usually diagnosed about 10 years following a breast augmentation or breast reconstruction procedure. 34
  • 36. Lymphoblastic Lymphoma (LL) Cells affected  immature  T- lymphocyte  T-LL  B-lymphocyte B-LL  It is one of the three most common types of NHL In children  Present in similar way to one another and similar to ALL  Age:0-15yrs –more common between 10-14 yrs. Types of Lymphoblastic Lymphoma (LL) 36
  • 37. T-Lymphoblastic Lymphoma (TLL)  TLL arise from precursor T lymphoblasts at different stages of differentiation  Consist 30% of all NHL of children  It is aggressive  Good prognosis 85% cured 37
  • 38.  consist about 80% of childhood LL sites:  thymus present as a mediastinal mass (mass behind the breast bone but in front of the windpipe, with breathing shortness) Precursor T-cell lymphoblastic Lymphoma 38
  • 39. 39
  • 40. Precursor B-cell lymphoblastic Lymphoma consist about 20% of childhood LL sites: Begins in lymph nodes outside the mediastinum (Neck, tonsils, or skin tumors) 40
  • 41. Symptoms of Lymphoblastic Lymphoma -LL the first symptoms notice a lump or several lumps, don't go away  sites of lumps; head, neck, chest, armpit or groin (are swollen lymph nodes)  advance stages: can spread to the lungs, (mediastinal mass) , liver, bones lumps 41
  • 42. the common symptoms of lymphoblastic lymphoma include painless swelling of lymph nodes easy bruising, persistence infections advance stages: can spread to the lungs, (mediastinal mass) shortness of breath, Cough, fatigue, prurittus 42
  • 43. Target for best treatmeant  Early stage (stage 1 and 2)  Advanced stage (stage 3 and 4) 43
  • 44.  Lymph node biopsy  B.M biopsy (Leukemia or Lymphoma) 25% or more blasts in the bone marrow  CBC, ESR, LFT  Chest X-ray, PET/CT positron emission tomography -PET 44
  • 45. Differences between Acute Lymphoblastic Leukemia vs Lymphoblastic Lymphoma  Are the same clinically and microscopicaaly 45
  • 46. 46