Pathology > Basic Hematology > White Cell Disorders > Lymphoma: Clinical- Hodgkin Lymphoma

Lymphoma: Clinical - Hodgkin Lymphoma

The clinical manifestations of Hodgkin lymphoma are, while not entirely unique, distinctive for the most part. Hodgkin lymphoma most commonly presents as painless enlargement of peripheral (superficial) lymph nodes. Cervical or supraclavicular lymph nodes are enlarged in as many as 80% of patients at presentation. Mediastinal involvement is common (10% initially; >50% later). In more than 90% of patients disease is located above the diaphragm at the time of initial diagnosis.

In most instances, Hodgkin lymphoma appears to spread or grow by contiguous extension (spreading from one lymph node to adjacent nodes or to extranodal tissue). Non-Hodgkin's lymphomas tend to be less defined in their growth pattern, commonly being diffuse, without evidence of contiguous spread.

The lymphadenopathy is often (approximately 25% of cases) accompanied by fever, night sweats (drenching the bedcloths or sheets), and weight loss (>10% in 6 months). Pruritus, usually generalized, is noted in about 10% of cases. An interesting, but rare symptom is pain in the involved lymph nodes secondary to alcohol ingestion.

The natural history of the disease correlates with the histopathology, the extent of disease and the presence or absence of symptoms* . The signs and symptoms are thus prognostically significant. It is for these reasons that patients are "staged".

Following the establishment of a diagnosis of Hodgkin lymphoma the patient undergoes a staging procedure which includes a physical examination, CT scan, laboratory tests (common findings in Hodgkin lymphoma are leukocytosis with lymphopenia,increased ESR, and anergy due to defective cellular immunity), and possible lymphangiogram, and in some cases laporotomy with splenectomy, liver and bone marrow biopsy.

* a - absence of symptoms; b - positive for systemic symptoms

 

"Ann Arbor" Staging Classification

 

Clinical staging includes a clinical history (special attention to "b" symptoms); a physical examination (special attention to lymph nodes, liver, spleen, and bone pain) and laboratory, roengenogram, total body CAT scan and possibly lymphangiogram examination.

Pathologic staging is the stage based on histopathologic information (other than the initial biopsy) obtained by exploratory laparotomy or by thoracotomy, bone marrow biopsy, or liver biopsy, etc.

Current recommended staging procedures (1992) include:

1) lymph node biopsy (not aspirate)
2) history and physical examination
3) chest roetgenogram
4) CAT scan of the chest,abdomen, and pelvis
5) laboratory tests (CBC-platelets & diff; ESR; liver function tests (LFTs)
6) lymphangiogram if CAT negative or laparotomy planned
7) staging laparotomy if not III b or IV or inoperable

Note: Laparotomy provides an accurate assessment of abdominal disease and avoids over or under treatment.

 

HEMATOPATHOLOGY

 

A twenty-five year old man comes to you complaining of a lump in his right neck. The lump has not changed since he first noticed it about six weeks ago. Lately he has been losing weight - 10 lbs. in the last month. His only medication is Tylenol for fever (101 ).

You obtain a chest X-ray and perform a lymph node and a bone marrow biopsy and touch prep of the marrow core.

With the results of this information you determine that this young man has:

A. stage IIIb, HD,nodular sclerosis type
B. stage IIb, HD, nodular scleosis type
C. stage IIIb, HD, lymphocyte predominant type
D. stage IIIa, HD, nodular sclerosis type
E. stage IVb, HD, nodular sclerosis type

 

A twenty-one year old woman comes to you complaining of night sweats in which her night cloths are soaking wet. Lately she has also noted dry skin and "gets real itchy after showering".

On physical examination you find a single, firm, rubbery left supraclavicular node. Her liver and spleen are normal to palpation. A chest roentgenogram showed mediastinal widening. An abdominal CAT scan was negative. A biopsy of the supraclavicular lymph node showed nodular sclerosing Hodgkin lymphoma.

With the results of this information you determine that this young woman has:

A. clinical stage Ib, HD,nodular sclerosis type
B. clinical stage IIa, HD, nodular scleosis type
C. clinical stage IIb, HD, nodular sclerosis type
D. clinical stage IIIa, HD, nodular sclerosis type
E. clinical stage IIIb, HD, nodular sclerosis type

 

Still working on the case of the twenty-one year old woman you elect to preform a laparotomy after a negative bone marrow biopsy . At laparotomy a 400 gram spleen was removed. Microscopic sections of the spleen revealed small nodules of Hodgkin lymphoma.

With the results of this information you now determine that this young woman has:

A. pathologic stage IIs,b HD,nodular sclerosis type
B. pathologic stage IIe,b HD, nodular scleosis type
C. pathologic stage IIIe,b HD, nodular scleosis type
D. pathologic stage IIIs,b, HD, nodular sclerosis type
E. pathologic stage IVb, HD, nodular sclerosis type

The discovery of disease in the spleen changes the stage from a Stage IIb to a Stage IIIs,b.

Approximately 20-30% of clinical stage I and II patients change to a pathologic stage III or IV with surgical staging.", " ../images/wcdgifs/hodgclinic3.gif" , "Answer")'></FORM>

 

 

A thirty-two year old, 125 lb. woman comes to you complaining of high fever, soaking night sweats and recent unexplained weight loss of about 15 lbs.

On physical examination you find a firm, rubbery left cervical node, but no hepatosplenomegaly. A chest roentgenogram showed mediastinal widening with involvement of the lung parenchyma by an infiltrative process. An abdominal CAT scan showed no lymphadenopathy. A biopsy of the left cervical lymph node was read as mixed cellularity Hodgkin lymphoma. Biopsy of the lung was positive for Hodgkin lymphoma.

With the results of this information you determine that this young woman has:

A. clinical stage IIb, HD, mixed cellularity type
B. clinical stage IIe,b, HD, mixed cellularity type
C. clinical stage IIs,b, HD, mixed cellularity type
D. clinical stage III,b, HD, mixed cellularity type
E. clinical stage IV,b, HD, mixed cellularity type

There is both supraclavicular and mediastinal disease (stage II) with extension (e) from the mediastinal nodes into the lung (stage IIe).
The presence of night sweats or fever or weight loss constitute "b" symptoms. There was no disease below the diaphragm therefore this is a stage IIe,b.", " ../images/wcdgifs/hodgclinic5.gif" , "Answer")'></FORM>

 

Lymphoma: Clinical - Hodgkin Lymphoma

 

Subtypes

Relative Frequency
5 Year Survival
Stage at Dx
"b" Symptoms at Dx

Lymphocyte predominant

5-l0%
90%

90% PS I-II

4%

Nodular sclerosis

50-70%
80%

60% PS I-II

34%

Mixed cellularity

20-30%
50-60%

54% PS III-IV

37%

Lymphocyte depletion

<5%
20%

77% PS III-IV

73%

modified Stanford Series

Hepatosplenomegaly is unusual at initial diagnosis, but when present generally indicate advanced disease.

Stages I & II generally have a better prognosis than stage III, which is in turn better than stage IV.

Younger patients have a better prognosis than older patients.

We have already seen that the presence of symptoms correlates with a poor prognosis and that the histopathology is an important predictor of prognosis.

 

HEMATOPATHOLOGY

 

A thirty-five year old man comes to you because of bone pain. He noticed a lump in his neck which increased in size since he first found it about three weeks ago. Lately he has been losing weight - 6 lbs. in the last month and over 15 lbs in the past 6 months. He has seen his physician for fever of > 100 on several occasions in the last month. A lymph node biopsy is positive for HD. Liver function studies are grossly abnormal. The PB smear shows a myeloid left shift (promyelocytes and myelocytes) and moderate numbers of NRBCs. Additional pathologic nodes are found in the inguinal region and abdominal lymphadenopathy is detected by CAT scan.

With the results of this information you determine that this young man most likely has:

A. stage IIIb, HD,nodular sclerosis type
B. stage IIb, HD, mixed cellularity type
C. stage IVb, HD, lymphocyte predominant type
D. stage IIb, HD, nodular sclerosis type
E. stage IVb, HD, lymphocyte depleted type

 

Lymphoma: Clinical - Hodgkin Lymphoma

 

As we said earlier the goal of the physician is to establish a diagnosis, determine the extent of disease (stage), and institute appropiate therapy for the patient.

The aim of treatment should be to eliminate known disease and potential disease (areas where disease is likely to next appear) while minimizing the amount of treatment and the side effects of treatment.

Early stage Hodgkin lymphoma is usually treated with radiation while chemotherapy + radiation is used in advanced disease or for salvage therapy.

Radiation therapy is carefully limited to the area of disease by limiting the radiation field or port to the region of known and potential disease based on the usual pattern of contiguous spread.

Staging laparotomy allows more accurate assessment of disease extent, avoiding the overtreatment of patients with chemotherapy and the undertreatment of patients with radiotherapy. Overall survival & cure rates are probably not increased. Furthermore, CT scan accuracy and the risks of splenectomy (sepsis & leukemia) restrict the use of laparotomy.

 

In Hodgkin lymphoma radiation therapy is usually delivered into three major ports:

1) mantle
2) para-aortic
3) pelvic (inverted "Y")

Mantle and para-aortic fields together are someyimes referred to as "extended field" radiation.

Mantle, para-aortic, and pelvic radiation together constitute total-nodal radiation therapy.

For stage I and II disease, the 5-year survival with radiation therapy only is 80-90 %.

 

The standard dose of radiation therapy is in the range of 3,200-

4,000 CGy. Common complications, depending on the field, include hypothyroidism, pericarditis, pneumonitis, pulmonary fibrosis, sterility (female), myelosuppression (leukopenia), esophagitis, and diarrhea.

What is the port at right called ?

 

Advanced or late stage disease is usually treated with combination chemotherapy "curing" more than 50 %.

Two of the best and most commonly used chemotherapy regimens against Hodgkin lymphoma are MOPP and ABVD. For a table of chemotherapy agents see the "Dictionary" button below.

The chemotherapy regimen usually involves 6-12 months of treatment.

Complications may limit chemotherapy.

Short-term side effects include: myelosuppression causing leukopenia, nausea, vomiting, alopecia, neuropathy, stomatitis, and opportunistic infections (Pneumocystis carinii; herpes).

Long-term effects include sterility (males>females), cardiomyopathy (Adriamycin), and secondary malignancies in about 5% (leukemia and non-Hodgkin lymphoma). The risk of second malignancies increases with time and may be as high as 20% at 15-20 years.

 

Radiation and chemotherapy have dramatically altered the outlook or prognosis for patients with Hodgkin lymphoma when compared to the natural course of the disease (30% 5-year survival in 1950). Although difficult to define "cure" we generally use the 5-year survival as the 'cure' rate. With each disease-free year, the probability of relapse decreases.

The prognosis for women may be better because of the higher proportion of nodular sclerosis HD in women.

Subtypes
5 Year Survival**
Median Survival

Lymphocyte predominant

90%
8 years

Nodular sclerosis

80%
4 years

Mixed cellularity

50-60%
2.5 years

Lymphocyte depletion

20%
1.5 years

** mean 5-year survival of 30% in 1950 improved to 74% in 1984

 

HEMATOPATHOLOGY

 

A twenty-five year old man comes to you because of a lump in his neck which has been present about three weeks without change. Lately he has lost weight - 15 lbs. in the last month. He has been seen in student health for fever of > 100 on several occasions in the last month. A cervical lymph node biopsy is positive for HD,nodular sclerosis type. Liver function studies are mildly abnormal. His chest roentgenogram is normal.

If clinical staging is that of stage Ib would a pathologic staging procedure in which the spleen and abdominal lymph nodes are positive, change the theraputic plan? If so, how?

A. no change in therapy, use radiation
B. no change in therapy, use chemotherapy
C. yes, change from radiation to chemotherapy
D. yes, change from chemotherapy to radiation
E. hold everything, there is no hope for this patient, DNR

A twenty-five year old man complains of a lump in his right neck. The lump has increased in size in the past seven weeks. He has lost weight - 12 lbs. in the last month and 20 in the last 6 months. His only medication is Tylenol for fever (101 ). He is HIV positive.

You obtain a chest roentgenogram which shows mediastinal widening and hilar lymphadenopathy. Lymph node and bone marrow biopsies are both positive for mixed cellularity Hodgkin lymphoma.

With the results of this information you make the following comments about HD in HIV+ patients, all of which are true EXCEPT for:

A. there is a higher incidence of HD compared to nonHIV+
individuals
B. HD is likely to present in advanced stages
C. mixed cellularity type is more common
D. extranodal sites of presentation are common
E. the clinical course is aggressive

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