A Cup of Coffee – Hodgkin’s Disease

Welcome back! Last week, we talked about a painful disorder called Endometriosis. If you missed that blog and would like to catch up, click HERE.

This week we are going to talk about a type of cancer called Hodgkin’s disease. I found out via research that Hodgkin’s disease has some rare syndromes inside of the original diagnosis. Confused yet? Yeah, I was too. Let’s chat.

The Technical Words

I understand that the audience I write for isn’t doctors. Neither am I. So I’m going to do my best to define words we aren’t normally exposed to as we go through this blog. If you, or someone you know, has Hodgkin’s disease, I’m going to do my best to help you understand today’s blog.

What is Hodgkin’s disease?

Let’s start here. According to NORD (National Organization of Rare Disorders), “Hodgkin’s disease is one of a group of cancers known as a lymphoma. Lymphoma is a general term used to describe cancers that affect the lymphatic system, especially the lymph nodes. Tumors often form in the lymph nodes (places where lymphatic vessels unite) and/or the area around the nodes. Fever, night sweats, and weight loss may occur along with swollen lymph nodes. The exact cause of Hodgkin’s disease is unknown.”

Types

There are several types of Hodgkin’s:

1. Chronic lymphocytic leukemia
2. Cutaneous B-cell lymphoma
3. Cutaneous T-cell lymphoma
4. Lymphoma

Symptoms

According to the Mayo Clinic, the following are common and not-so-common symptoms:

Signs and symptoms of Hodgkin’s lymphoma may include:

• Painless swelling of lymph nodes in your neck, armpits, or groin
• Persistent fatigue
• Fever
• Night sweats
• Losing weight without trying
• Severe itching
• Pain in your lymph nodes after drinking alcohol

Science Direct will help us break down the symptoms both common and rare.

Itching

Hodgkin’s disease (HD) causes itching in 10%—25% of patients. In some instances, itching precedes the diagnosis of lymphoma and may be an indicator of a less favorable prognosis when associated with significant fever or weight loss. Itching associated with HD is characterized by symptoms of burning and intense itching occurring on a localized skin area, frequently on the lower legs.

Other lymphomas and leukemias have been associated with less intense but more generalized itching. The itching may appear as a mild localized symptom, but if due to HD, it commonly progresses and becomes generalized.

Initially, there are no associated skin lesions, but with chronic excoriations (a wearing off of the skin) secondary changes of thickening, infections, and pigmentation may be seen. The itching may be the only systemic symptom (relating to a system, especially as opposed to a particular part) in the otherwise typical presentation, especially in women.

Itching alone does not worsen the prognosis of HD; this is the case only if this symptom is associated with other systemic signs.

Alcohol-Related Pain

Pain in one or more chains of enlarged lymph nodes almost immediately after ingestion of alcohol is a dramatic symptom that may occasionally be the presenting manifestation in HD. Although it appears to have some degree of specificity (the extent to which a diagnostic test is specific for a particular condition) for this disease, the fact that it occurs in a small minority of cases severely limits its diagnostic value.

It has been suggested that the alcohol intolerance syndrome in HD may be disappearing, perhaps reflecting a change in the natural history of the disease. While the incidence between 1950-1960 was reported to be around 15%, recent data from Stanford report a frequency of this symptom only in the order of l%-2%.

The decreasing incidence of this symptom complex may reflect the fact that an increasing proportion of HD cases is now detected at an earlier stage in the evolution of the disease. The pain associated with alcohol ingestion varies greatly in character and severity; some patients describe it as sharp and stabbing, others as dull and aching, or merely as malaise. In any case, the association with alcohol intake is usually clear enough to lead the patient to discontinue the use of alcohol in any form.

Commonly, the pain begins within a few minutes after an alcoholic drink is swallowed, and lasts anywhere from a few minutes to a few hours. An interesting feature of the pain is that it is usually experienced in the immediate vicinity of one or more sites of clinically evident lymphadenopathy. Pain has been observed to disappear after successful treatment and to signal an impending relapse in patients in whom other manifestations of the recurrent disease have not yet become clinically apparent.

Nephrotic Syndrome

Nephrotic syndrome = A kidney disorder that causes the body to excrete too much protein in the urine.

Hodgkin’s disease represents the neoplasm (a new and abnormal growth of tissue in some part of the body) most commonly associated with nephrotic syndrome.
Membraneous glomerulopathy (a type of glomerular disease – relating to a cluster of nerve endings, spores, or small blood vessels, especially around the end of a kidney tubule – and is an autoimmune disease) is reported in about 10% of nephrotic patients with HD, in contrast to 80%-90% of nephrotic patients with carcinoma (Carcinoma is cancer that forms in epithelial tissue. Epithelial tissue lines most of your organs, the internal passageways in your body – like your esophagus – and your skin. Most cancers affecting your skin, breasts, kidney, liver, lungs, pancreas, prostate gland, head, and neck are carcinomas).

The nephrotic syndrome in HD is rare, with an incidence of 0.4 % in combined series of 1700 cases. Age ranges from 6 to 60 years; all stages of the disease.

The reversal of the nephrotic syndrome has been observed in almost all instances where direct treatment has resulted in a complete remission of the disease. Recurrences of tumors have been associated with relapse of the nephrotic syndrome.

Hemolytic anemia

Hemolytic anemia = is a blood condition that occurs when your red blood cells are destroyed faster than they can be replaced.

It is sometimes difficult to distinguish the effects of therapy for HD from the effects of the disease itself on haematologic bases (relating to blood and the body tissues that make it).

Both radiotherapy and chemotherapy used in the management of HD may have profound acute and chronic effects on bone marrow function.

Anemia of a mild degree may be found in patients who present with widespread disease, often associated with systemic symptoms. This is usually anemia with normal indices (an indicator, sign, or measure of something), a normal or low reticulocyte count (Reticulocytes are red blood cells that are still developing), and a negative Coombs test.

The Coombs test checks your blood for antibodies that attack red blood cells. This test may be used to screen your blood before a procedure, such as a blood transfusion. Or, it may be used to find out if you have certain conditions, such as autoimmune hemolytic anemia.

Excluding the effects of therapy, some patients developed severe anemia later on. This may be due to extensive bone-marrow involvement, hypersplenism (an overactive spleen), or, rarely, a Coombs-positive hemolytic picture.

At diagnosis less than 1% of the patients will present with hemolytic anemia; later on, the overall incidence might rise to 2%-3%.

Recently, the antibody responsible for this hemolysis has been characterized as being an immunoglobulin G with anti-I1 specificity. This antibody may be unique for Coombs test-positive hemolytic anemia associated with HD.

Idiopathic Thrombocytopenia (ITP)

Idiopathic thrombocytopenia purpura (ITP) = Low levels of the blood cells that prevent bleeding (platelets).

Idiopathic thrombocytopenia purpura is uncommonly associated with HD.

ITP usually occurs either at the time of diagnosis or later. ITP associated with HD appears to be more severe and resistant to treatment than ITP alone or in association with other diseases.

Only 25% of the patients responded to steroids, although 50% of patients who had undergone splenectomy (the removal of your spleen) specifically for ITP appeared to have a good and durable response.

ITP can also occur in patients who have undergone splenectomy for staging purposes. This finding indicates that the antibody responsible for thrombocytopenia can be produced in sites other than the spleen.

Most patients develop ITP while in remission after successful treatment, and the occurrence of ITP does not necessarily indicate relapse. Because most reported cases occurred after splenectomy, the combination of corticosteroids and immunosuppressive drugs is required for treatment, and most patients respond well to this treatment.

Bone Lesions

Bone lesions = any process that replaces normal healthy bone with abnormal bone or tissue.

Bone lesions, generally isolated, are very rare at diagnosis and are seen in less than 1% of patients.

Isolated bone lesions do not change long-term disease-free survival if treated appropriately with radiation therapy. Thus, involvement of the bone should not be equated with involvement of the marrow unless there is other evidence of widely disseminated disease.

Later in the course of the disease, involvement of the bone becomes more common, so that in the end about 10% of patients demonstrate some bone lesions.

In 75% of the cases, these are lytic ( Destruction of an area of bone due to a disease process, such as cancer), in less than 20% sclerotic (an unusual hardening or thickening of your bone), while 5% of the cases have mixed lesions.

There are two mechanisms leading to bone involvement: one is through the hematogenous spread (Originating in the blood or spreading through the bloodstream) and the other is per continuitatem (denoting the mode by which inflammation or other morbid process spreads from one part to another through continuous tissue), generally from paravertebral(situated, occurring, or performed beside or adjacent to the spinal column) lymph nodes.

In this case, the so-called phenomenon of ‘ivory vertebrae’ may be present. The finding of an ivory vertebra means that one or more vertebrae have been affected by a density increase without any changes in the opacity or size of the adjacent intervertebral discs.

CNS Involvement

CNS involvement = Central Nervous System; consists of the brain and spinal cord.

Neurologic manifestations are very rare in HD. They are mainly due to direct CNS involvement, but
can also be the expression of a paraneoplastic syndrome (a group of rare disorders that occur when the immune system has a reaction to a cancerous tumor known as a “neoplasm.”) or of an infectious complication (e.g., brain toxoplasmosis).

Neurologic paraneoplastic syndromes are most often caused by ‘autoimmune reactions’ with the presence of antineuronal antibodies. Antineuronal antibodies serve as markers that aid in discriminating between a true paraneoplastic neurological disorder (PND) and other inflammatory disorders of the nervous system.

Direct CNS involvement in HD is exceedingly rare, with only 48 cases described in the English literature up to 1995 and an overall incidence of 0%- 0.2% in the published series. CNS invasion may be more common in HD secondary to acquired (AIDS) or congenital immune deficiency, or in familial HD (in which immunodeficiency is common).

The finding of a primary solitary intracranial lesion at presentation is very uncommon, and CNS involvement in HD is usually related to disease relapse or progression and very often is documented only after death during autopsy.

Dural involvement (The tough outer layer of tissue that covers and protects the brain and spinal cord and is closest to the skull) is more common than parenchymal lesions (abnormalities of vascular origin), and the predominant histologic subtype is nodular sclerosis present in more than half of cases.

When combined modality treatment (neurosurgery if indicated, irradiation, and chemotherapy) is given with curative intent, prolonged disease-free survival may be achieved.

Conclusion

We have discussed seven rare syndromes which might be present at diagnosis and/or developed later on during the course of the disease. These syndromes show a decreasing incidence, which is most probably linked to an earlier diagnosis and better therapeutic results.

These syndromes remain nevertheless important since they are intimately correlated with the pathophysiology of Hodgkin’s disease.

If you, or someone you know, think they may have any of these symptoms, they should contact their doctor for a consult. The internet is no place for a diagnosis.

You did it!

If you got this far, go you! I know this was a tough blog to slog through, but it had a lot of good information for those dealing with this disease.

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As always, this blog is not a replacement for sound medical advice. I am not a doctor. Please make an appointment to see your healthcare provider and put a good plan in place that works for you and the needs of your body.

That’s all I have for you this week, dear reader. I’ll see you back here next Wednesday to share another cup of coffee. Until then, be good to yourself and each other.

Mind, Body, Spirit…Osteopathic Doctors treat the whole person, not just the ailment. Is your PCP a DO? Would you like to learn more about Osteopathic Physicians? Click HERE!