What is Richter’s Syndrome & How is it Treated? | Causes, Symptoms, Diagnosis, Outlook of Richter’s Syndrome

Richter’s syndrome is the rapid progression of chronic lymphocytic leukemia into a more aggressive form of lymphoma.

Chronic lymphocytic leukemia is a type of cancer that affects white blood cells. Richter’s syndrome is quickly transforming of chronic lymphocytic leukemia into an aggressive form of lymphoma.

Richter’s syndrome is transformed into diffuse large B-cell lymphoma and in rare cases, it may transform into Hodgkin lymphoma. It is often referred to by experts as Richter transformation.

A study from 2022 noted surveillance data of epidemiology. It was observed that amongst over 74,000 people with chronic lymphocytic leukemia, the transformation rate was 0.7%.⁽¹⁾

Richter’s syndrome was associated by health experts with a negative outlook. According to a study done in 2020 on 200 people, individuals with the best outlook had cases of untreated chronic lymphocytic leukemia before a diagnosis of Richter’s syndrome.

The exact cause of why Richter’s syndrome occurs is not known but several factors are believed to be involved in it, including:

• Specific gene mutations
• Clinical features and biological characteristics of CLL cell
• Certain inherited genetic characteristics including BCL-2, LRP4, or CD38

The risk factors of Richter’s syndrome are similar to the potential causes. There are certain features of tumor cells that can be potential risk factors. Loss of certain chromosomes in tumor cells or atypical proteins in tumor cells might play a role in increasing a person’s risk of developing the condition.

There are several symptoms that may be seen due to Richter’s syndrome. These include:⁽²⁾

• A rapid rise in the size of the lymph nodes in the neck, abdomen, armpit or groin.
• Night sweat
• Weight loss
• Fever

Additional signs and symptoms include:

• Palpitations
• Fatigue
• Shortness of breath
• Low platelet count
• Dizziness

An increase in the level of serum lactate dehydrogenase and serum calcium may be observed in the routine blood work.

The symptoms of Richter’s syndrome may mimic other noncancerous conditions. Therefore, it is important for a person to consult a healthcare professional to diagnose the reason for its occurrence.

Richter’s syndrome may be suspected if a person has or was previously diagnosed with chronic lymphocytic leukemia.

A PET scan is done which may be helpful in determining the best lymph nodes for biopsy.

Also, if Richter’s syndrome is suspected, a surgeon is asked to perform a biopsy or CT scan of the enlarged lymph node. The biopsy can be helpful in determining the typical progression of chronic lymphocytic leukemia or Richter’s syndrome development.

The treatment may depend on the subtype of Richter’s syndrome. The majority become diffuse large B-cell lymphoma. The standard treatment for this type may involve chemotherapy and immunotherapy.⁽²⁾

Those with Hodgkin lymphoma may receive chemotherapy as the first line of treatment without immunotherapy.

These therapies may be helpful in short-term remission and are also linked with potential side effects.⁽³⁾ If a person achieves remission, the doctor may perform a stem cell transplant. This has long-term survival associated with it.

The outlook of Richter’s syndrome is described as negative by doctors.⁽¹⁾ Here in the report, the median survival rate is 10 months. The relationship between the transformed diffuse large B-cell lymphoma and the underlying chronic lymphocytic leukemia is the most important aspect of the outlook.

A person who goes into remission and receives a stem cell transplant may have a better outlook.⁽³⁾

Researchers are continually looking into new treatments to improve survival rates.

Richter’s syndrome is the transformation of chronic lymphocytic leukemia into an aggressive lymphoma, which has a negative outlook. Enlargement of the lymph nodes is the most common symptom. It is important to be in touch with a doctor to get appropriate treatment. The outlook is better for those whose disease goes into remission and who undergo stem cell therapy.