Acute:
Haematology: Cancers - Early stages: 1 or 2 ABV
Lymphoma dacarbazine
Leukaemias - Advanced: 3 or 4 AVD a
MGUS Or: BEACOPP: bleomycin,
vincristine, procarbazine,
Multiple myeloma - Asymptomatic pts:
IA, IIA: rituximab and chem
III, IV: rituximab and chem
1. Lymphoma: Hodgkin’s Chronic pts:
Mostly presents with: Painless cervical and/ or supraclavicular lymphadenopathy - See local specialist guideli
in young adults - Salvage therapy combined
B symptoms: fever (temp >38), night sweats, weight loss >10% in the last 6 - Stem cell transplantation
months - Consider brentuximab
malignant Reed-Sternberg cells harbour Epstein-Barr virus (EBV) antigens in a
significant proportion of cases (20% to 40%)
2. Non-Hodgkin's lymph
RFs:
Hx of mononucleosis is a risk factor for EBV-positive Hodgkin lymphoma
- EBV with Burkitt’s lympho
It is a B-cell malignancy
- EBV with AIDS related prim
- EBV with nasal natural kill
Other signs and symptoms:
- Hep C with splenic margin
- Dyspnoea
- Hep C with diffuse large B
- Cough
- Human herpesvirus 8 with
- Chest pain
- H.pylori with gastric MALT
- Asymmetric lymphadenopathy
- Borrelia burgdorferi with
- SVC syndrome cough, SOB, face and upper extremity oedema, dilated
- Coxiella burnetiid with B c
neck veins
- Chlamydia psittaci in MAL
- Abdominal pain
- Pruritus
Associated with:
- ** alcohol-induced pain at involved sites
- Sjogren syndrome, rheum
- Hepatosplenomegaly
- Post organ transplant and
- Tonsillar enlargement
- Ataxia telangiectasia, Kline
- Pesticides and phenoxyhe
Classification:
- Breast implants increase r
Type Frequency Prognosis Notes
Nodular sclerosing Most common: Good More in women
B-cell lymphoma:
70% Linked to lacunar
- immature B cells, mature
cells
B cells may transform to v
Mixed cellularity 20% Good Large number of
diffuse large B-cell lympho
Reed Sternberg
T- cell lymphoma:
cells
- Pluripotent haematopoieti
Lymphocyte 5% Best - and T)
predominant
- T cells migrate early into t
Lymphocyte Rare Worst - the process starts from th
depleted (TCR) alpha, forming the m
CD4+/CD8+
factors associated with a poor prognosis:
- Age >45 Classifications:
- Stage 4 disease 1. Mature B cell neoplasm:
- Hb<10.5 CLL/ small lymphocytic lym
- Lymphocyte count <600/ microL or <8% Monoclonal B cell lympho
- Male Splenic marginal zone lym
- Albumin <40 g/l B cell prolymphocytic leuk
- WBC > 15000/ microL Hairy cell leukaemia
Splenic B cell lymphoma/
Lymphoplasmacytic lymph
Staging: Ann-Arbor staging MGUS IgM: monoclonal g
1. Single lymph node Heavy chain diseases: alph
2. 2 or more lymph nodes/ regions on the same side of the diaphragm disease
3. Nodes on both sides of the diaphragm MGUS IgA/ IgG
4. Beyond lymph nodes Plasma cell myeloma
Divide each stage into A or B: Solitary plasmocytoma of
A: no systemic symptoms other than pruritus Extraosseous plasmacytom
B: having B symptoms Follicular lymphoma in
Paeds type of follicular lym
Risk factors: Large B cell lymphoma
, Mantle cell Usually, B lymphocytes
High-grade B-cell lymphoma, not otherwise specified Leading to pancytopenia
B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and Mostly in children and als
classical Hodgkin's lymphoma older adults
More common in Down
b. Aggressive T-cell lymphomas Associated with
Philadelphia chromosom
Enteropathy-type T-cell lymphoma/intestinal T-cell lymphoma Peak incidents: age <5
Peripheral T-cell lymphoma
Subcutaneous panniculitis-like RFs:
Systemic anaplastic - Hx malignancy
Angioimmunoblastic - Viral infections
- Hx of chemotherapy
c. Indolent B-cell lymphomas - Radiation exposure and sm
- Down syndrome and Kline
Follicular lymphoma telangiectasia
Marginal zone; gastric MALT type - Male
Chronic lymphocytic leukaemia/small lymphocytic lymphoma - Hispanic population
Lymphoplasmacytic lymphoma - FHx
o Waldenström's macroglobulinaemia
d. Indolent T-cell lymphomas Signs and symptoms:
- Fever
Mycosis fungoides/Sézary syndrome - Pallor
Primary cutaneous anaplastic large cell lymphoma - Fatigue and dizziness
- Lymphadenopathy
SIGNS AND SYMPTOMS: - Hepatosplenomegaly
- B symptoms - petechiae
- Fatigue - bruising and epistaxis and
- Lymphadenopathy - bone pain
- Splenomegaly - renal enlargement
- Hepatomegaly - meningismus and papilloe
- Cough - abdominal pain
- SOB - mediastinal and/or abdom
- Headache - pleural effusion
- Abdominal discomfort
- Chest pain Investigate:
- Pallor FBC
- Purpura Blood smears
- Jaundice Electrolytes Ca can be high
- Skin lesion Uric acid: high: due to rapid tu
- Neurological sign Tumour lysis syndrome:ONCO
hyperphosphatemia, hyperkal
Investigations: hyperuricemia and high LDH
- FBC thrombocytopenia, pancytopenia, lymphocytosis Renal function
- Blood smears: early WBC precursor, nucleated RBC LFT
- Bone marrow biopsy Coagulation profile
- LFT Bone marrow aspiration hyp
- Metabolic panel leukemic lymphoblasts >20% b
- LDH: determining the urgency of treatment and intensity of Tumour lysis Immunophenotyping
prophylaxis Cytogenetic analysis Philad
- Lymph node biopsy Blood group and antibody scre
- PET/CT Antibodies for infections HI
- Skin biopsy HLA typing
- Immunoglobulin gene rearrangement study CXR
- FISH and cytogenetic study Lumbar puncture
- HIV antibody Pleural tap
- Hep B and C CT/MRI brain
- Lumbar puncture low glucose and high protein and pressure CT chest
- Polymerase chain reaction for tumour marker **scrotal US
- Serum protein electrophoresis
- +/- Echocardiogram and colonoscopy Management:
- ACUTE PHASE:
Management: a. Induction th
Watchful waiting cyclophosph
Chemo daunorubicin
Monoclonal antibodies b. Supportive c
Haematology: Cancers - Early stages: 1 or 2 ABV
Lymphoma dacarbazine
Leukaemias - Advanced: 3 or 4 AVD a
MGUS Or: BEACOPP: bleomycin,
vincristine, procarbazine,
Multiple myeloma - Asymptomatic pts:
IA, IIA: rituximab and chem
III, IV: rituximab and chem
1. Lymphoma: Hodgkin’s Chronic pts:
Mostly presents with: Painless cervical and/ or supraclavicular lymphadenopathy - See local specialist guideli
in young adults - Salvage therapy combined
B symptoms: fever (temp >38), night sweats, weight loss >10% in the last 6 - Stem cell transplantation
months - Consider brentuximab
malignant Reed-Sternberg cells harbour Epstein-Barr virus (EBV) antigens in a
significant proportion of cases (20% to 40%)
2. Non-Hodgkin's lymph
RFs:
Hx of mononucleosis is a risk factor for EBV-positive Hodgkin lymphoma
- EBV with Burkitt’s lympho
It is a B-cell malignancy
- EBV with AIDS related prim
- EBV with nasal natural kill
Other signs and symptoms:
- Hep C with splenic margin
- Dyspnoea
- Hep C with diffuse large B
- Cough
- Human herpesvirus 8 with
- Chest pain
- H.pylori with gastric MALT
- Asymmetric lymphadenopathy
- Borrelia burgdorferi with
- SVC syndrome cough, SOB, face and upper extremity oedema, dilated
- Coxiella burnetiid with B c
neck veins
- Chlamydia psittaci in MAL
- Abdominal pain
- Pruritus
Associated with:
- ** alcohol-induced pain at involved sites
- Sjogren syndrome, rheum
- Hepatosplenomegaly
- Post organ transplant and
- Tonsillar enlargement
- Ataxia telangiectasia, Kline
- Pesticides and phenoxyhe
Classification:
- Breast implants increase r
Type Frequency Prognosis Notes
Nodular sclerosing Most common: Good More in women
B-cell lymphoma:
70% Linked to lacunar
- immature B cells, mature
cells
B cells may transform to v
Mixed cellularity 20% Good Large number of
diffuse large B-cell lympho
Reed Sternberg
T- cell lymphoma:
cells
- Pluripotent haematopoieti
Lymphocyte 5% Best - and T)
predominant
- T cells migrate early into t
Lymphocyte Rare Worst - the process starts from th
depleted (TCR) alpha, forming the m
CD4+/CD8+
factors associated with a poor prognosis:
- Age >45 Classifications:
- Stage 4 disease 1. Mature B cell neoplasm:
- Hb<10.5 CLL/ small lymphocytic lym
- Lymphocyte count <600/ microL or <8% Monoclonal B cell lympho
- Male Splenic marginal zone lym
- Albumin <40 g/l B cell prolymphocytic leuk
- WBC > 15000/ microL Hairy cell leukaemia
Splenic B cell lymphoma/
Lymphoplasmacytic lymph
Staging: Ann-Arbor staging MGUS IgM: monoclonal g
1. Single lymph node Heavy chain diseases: alph
2. 2 or more lymph nodes/ regions on the same side of the diaphragm disease
3. Nodes on both sides of the diaphragm MGUS IgA/ IgG
4. Beyond lymph nodes Plasma cell myeloma
Divide each stage into A or B: Solitary plasmocytoma of
A: no systemic symptoms other than pruritus Extraosseous plasmacytom
B: having B symptoms Follicular lymphoma in
Paeds type of follicular lym
Risk factors: Large B cell lymphoma
, Mantle cell Usually, B lymphocytes
High-grade B-cell lymphoma, not otherwise specified Leading to pancytopenia
B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and Mostly in children and als
classical Hodgkin's lymphoma older adults
More common in Down
b. Aggressive T-cell lymphomas Associated with
Philadelphia chromosom
Enteropathy-type T-cell lymphoma/intestinal T-cell lymphoma Peak incidents: age <5
Peripheral T-cell lymphoma
Subcutaneous panniculitis-like RFs:
Systemic anaplastic - Hx malignancy
Angioimmunoblastic - Viral infections
- Hx of chemotherapy
c. Indolent B-cell lymphomas - Radiation exposure and sm
- Down syndrome and Kline
Follicular lymphoma telangiectasia
Marginal zone; gastric MALT type - Male
Chronic lymphocytic leukaemia/small lymphocytic lymphoma - Hispanic population
Lymphoplasmacytic lymphoma - FHx
o Waldenström's macroglobulinaemia
d. Indolent T-cell lymphomas Signs and symptoms:
- Fever
Mycosis fungoides/Sézary syndrome - Pallor
Primary cutaneous anaplastic large cell lymphoma - Fatigue and dizziness
- Lymphadenopathy
SIGNS AND SYMPTOMS: - Hepatosplenomegaly
- B symptoms - petechiae
- Fatigue - bruising and epistaxis and
- Lymphadenopathy - bone pain
- Splenomegaly - renal enlargement
- Hepatomegaly - meningismus and papilloe
- Cough - abdominal pain
- SOB - mediastinal and/or abdom
- Headache - pleural effusion
- Abdominal discomfort
- Chest pain Investigate:
- Pallor FBC
- Purpura Blood smears
- Jaundice Electrolytes Ca can be high
- Skin lesion Uric acid: high: due to rapid tu
- Neurological sign Tumour lysis syndrome:ONCO
hyperphosphatemia, hyperkal
Investigations: hyperuricemia and high LDH
- FBC thrombocytopenia, pancytopenia, lymphocytosis Renal function
- Blood smears: early WBC precursor, nucleated RBC LFT
- Bone marrow biopsy Coagulation profile
- LFT Bone marrow aspiration hyp
- Metabolic panel leukemic lymphoblasts >20% b
- LDH: determining the urgency of treatment and intensity of Tumour lysis Immunophenotyping
prophylaxis Cytogenetic analysis Philad
- Lymph node biopsy Blood group and antibody scre
- PET/CT Antibodies for infections HI
- Skin biopsy HLA typing
- Immunoglobulin gene rearrangement study CXR
- FISH and cytogenetic study Lumbar puncture
- HIV antibody Pleural tap
- Hep B and C CT/MRI brain
- Lumbar puncture low glucose and high protein and pressure CT chest
- Polymerase chain reaction for tumour marker **scrotal US
- Serum protein electrophoresis
- +/- Echocardiogram and colonoscopy Management:
- ACUTE PHASE:
Management: a. Induction th
Watchful waiting cyclophosph
Chemo daunorubicin
Monoclonal antibodies b. Supportive c
