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Hodgkin’s lymphoma

Hodgkin’s lymphoma . Rakesh Biswas MD, Professor, Department of Medicine, People's College of Medical Sciences, Bhanpur, Bhopal, India.

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Hodgkin’s lymphoma

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  1. Hodgkin’s lymphoma Rakesh Biswas MD, Professor, Department of Medicine, People's College of Medical Sciences, Bhanpur, Bhopal, India

  2. A 25 year old lady1 month of evening rise of temperature, night sweats and noticed a lump in her neckOn examination pallor, generalized lymphadenopathy, hepatosplenomegaly

  3. I wasn't feeling well, still couldn't shake the cold that had been plaguing me for what seemed like months. But all in all, not feeling too bad, either.

  4. It is required that anybody teaching in the schools have a clear tuberculosis record. After waiting several minutes, the nurse read my test site. She said it seemed to her there was a little swelling that shouldn't be there, and she asked me to wait and see the doctor.

  5. I started to get a little bit nervous, I mean, Tuberculosis? Was that possible?The doctor brought me into her room and she examined me, and she said she could feel tumors in my neck. Had I noticed them?

  6. Lymphoma • Clonal malignant disorders that are derived from lymphoid cells: either precursor or mature T-cell or B-cell • Majority are of B- cell origin • Divided into 2 main types : 1. Hodgkin’s lymphoma 2. Non - Hodgkin’s lymphoma

  7. Hodgkin’s Disease • Histologically & clinically a distinct malignant disease • Predominantly, B-cell disease • Course of the disease is variable, but the prognosis has improved with modern treatment

  8. Etiology • ? Infection – EBV • ? Environmental factors

  9. REAL* Classification • Classic: • Nodular Sclerosis • Lymhocyte rich • Mixed Cellularity • Lymhocyte depleted • Non-Classic • Nodular Lymphocyte predominant *REAL – Revised European,American,lymphoma

  10. Clinical features • Bimodal agedistribution : • young adults ( 20-30 yrs) & elderly (> 50yrs) May occur at any age • M > F • Lymphadenopathy: • most often cervical region • asymmetrical, discrete • painless, non-tender • elastic character on palpation ( rubbery) • not adherent to skin • fluctuate in size

  11. Contiguous spread via the lymphatic chain eg.involvement of abdominal & thoracic LNs Extra nodal disease - rare Hepatospleenomegaly

  12. Constitutional symptoms ( B symptoms ) Night sweats, sustained fever > 38 degree celsius, loss of weight >10% of body weight in 6 mo Fever sometimes cyclical (‘Pel-Ebstein fever’) Pain at the site of disease after drinking alcohol Pallor Pruritis Symptoms of Bulky (>10 cm) disease

  13. A zillion tests were done, blood drawn a zillion times, a zillion questions by a million doctors. Finally it was decided they would have to perform a biopsy on one of the tumors to get a diagnosis.

  14. Investigations • CBP : Anemia ( normochromic / normocytic), eosinophilia, neutrophilia, lymphopenia • ESR -raised • LFT- (liver infil / obs at porta hepatis) • RFT- prior to treatment • Urate , Ca, • LDH - adverse prognosis • CXR- mediastinal mass • CT thorax / abdomen / pelvis-for staging • Other: Gallium scan, PET, Lymphangiography , Laporotomy

  15. LN FNAC / biopsy : Malignant REED-STERNBERG ( RS) Cell: Bi-nucleate cell with a prominent nucleolus. Derived from B cell, at an early stage of differentiation Reactive background of eosinophils, lymphocytes, plasma cells Fibrous tissue

  16. The operation was done on February third, my boyfriend's birthday. I made him a card out of paper I had…Hard to celebrate your boyfriend's birthday while getting ready to get operated on.

  17. REED-STERNBERG ( RS ) Cell

  18. REED-STERNBERG ( RS) Cell

  19. The X-ray technician came out again, looking for me. "We're not ready for you yet, the Doctors are still reading your chart, but we wanted to make sure you didn't leave. Just wait a few minutes and the doctor will be out to talk to you."

  20. I've read in novels the expression "my heart sank" but I'm not sure I ever really felt that sort of thing until just about then. I'd been telling myself there was nothing to worry about, all was ok, but this was a clear cut sign that something was very. very wrong

  21. >10 cm Bulky disease

  22. Lymphangiography

  23. Staging • Stage I : Involvement of single LN region (I) or extra lymphatic site (IAE ) • Stage II :Two or more LN regions involved (II) or an extra lymphatic site and lymph node regions on the same side of diaphragm • Stage III : Involvement of lymph node regions on both sides of diaphragm, with (IIIE) or without (III) localized extra lymphatic involvement or involvement of the spleen (IIS) or both (IISE) • Stage IV :Involvement outside LN areas (Liver, bone marrow) A : Absence of ‘B’ symptoms B : B symptoms present

  24. I had Stage IVb Hodgkin's Disease, a form of cancer of the lymph nodes.Well, it's sort of hard to describe what hearing something like that does. It seemed impossible that the nodes in my neck, and the masses that were supposedly on my lungs were cancer. How could that be? I just was amazed. I said "this is unbelievable." and felt more or less detached, numb.

  25. Treatment • RT • Chemo • BMT / SCT • Antibody treatment: Rituximab target CD-20 • Supportive

  26. Treatment - Guidelines • Indications for RT: • Stage I disease • Stage II disease with 3 or lesser areas involved • For Bulky disease • For pressure problems • Indications for CT • All with B symptoms • Stage II disease with >3 areas involved • Stage III and IV disease

  27. Treatment • Stage IA , Stage IIAwith3 or < 3 areas involved: Radiotherapy • Stage IB, Stage II A with > 3 areas , Stage IIB: Chemotherapyevery 3-4 weeks, 6-8 cycles; either alone, or in combination with radiotherapy • Stage III & IV :Chemotherapy + Radiotherapy ( for bulky disease or palliation of symptoms)

  28. Irradiation fields used in Hodgkin’s Lymphoma

  29. Chemotherapy • MOPP :Nitrogen Mustard, Vincristine (Oncovin), Procarbazine, Prednisolone • ABVD:Adriamycin, Bleomycin, Vinblastine, Dacarbazine • Higher dose for relapse or younger pts with poor prognostic features

  30. After six cycles of chemotherapy, my CT scans still show masses on my chest and in my neck. On July 7, I had a meeting with my doctors, and was told that if I continued with standard chemotherapy, my chances of being cured stand at less than ten per cent.

  31. Prognosis • Overall 10 yr survival – 80% • In long term survivors there is a risk of • secondary malignancy: (leukemia , NHL), Solid tumors- Lung, breast • Infections • Cardiac, pulmonary, endocrinal abnormalities

  32. International Prognostic Index (IPI) • Age • Advanced stage disease • Performance status • Elevated LDH • Presence of Extra nodal disease

  33. Non Hodgkin’s lymphoma • Incidence is increasing • NHL>HD • Median age of presentation is 65-70 yrs • M>F • More often clinically disseminated at diagnosis • B-cell-70% ; T-cell-30%

  34. ‘1990…Although I had been feeling fine, no different from normal, I was worried about this lump in my neck that I had for several months. I first thought it was just because I had some sort of infection, but it didn't go away.

  35. Clinical features • Widely disseminated at presentation • Nodal involvement: Painless lymphadenopathy, often cervical region is the most common presentation • Hepatospleenomegaly • Extranodal : Intestinal lymphoma( abdominal pain, anemia, dysphagia); CNS( headache, cranial nerve palsies, spinal cord compression) ; Skin, Testis; Thyroid; LungBone marrow(low grade): Pancytopenia

  36. Systemic symptoms • Sweating, weight loss, itching • Metabolic complications:hyperuricemia, hypercalcemia, renal failure • Compression syndrome: • Gut obstruction • Ascites • SVC obstruction • S/C Compression

  37. 'The surgeon took a biopsy of the lump, taking a few cells out with a needle to be looked at under the microscope. When the results came back a few weeks later, he told me that they showed I had non-Hodgkin's lymphoma

  38. Diagnosis and staging • Similar to HD plus, • Bone marrow aspirate & trephine • Immunophenotyping : Monoclonal antibodies directed against specific lymphocyte associated antigens B cell antigens ( CD 19, 20, 22); T cell antigens ( CD 2, 3, 5 & 7) • Immunoglobulin determination: Ig G / IgMpraprotein marker • HIV • CT / MRI

  39. Classification • REAL • Clinical / Working Formulation • Low grade • Inermediate grade • High grade

  40. Low grade Proliferation: Low Course: Indolent Symptoms: -ve Treatment: Not curable High grade High Rapid, fatal(un-Rx) +ve Potentially Curable Classification Staging Similar to HD

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