Erdheim-Chester Disease
Description
Patient Information
Age/Sex: female, 56 years old
Job Title: Instructor
Previous medical history: retroperitoneal tumor biopsied (no diagnosis at first); mesenteric panniculitis discovered eight years ago
Personal/Family History: No substantial family history, no smoking or alcohol use, and no hazardous exposure
Clinical Program
First Presentation: Eight years ago
large lump in the retroperitoneum
Biopsy: foamy cell histiocytic growth →steroid treatment → no change, bulk stable
Subsequent Events:
2004: Inflammatory cell-filled pericardial effusion → no diagnosis
In 2005, lost to follow-up
Present symptoms (at the time of admission):
Four weeks of slurred speech and an increasingly shaky pace
Constipation, exhaustion, swollen cheeks, nocturnal sweats, and a deep, unfluent voice
Physical Assessment
Vitals normal, afebrile
Neurological: clonus, Babinski sign, hyperreflexia, and cerebellar ataxia
Additional results:
Hypertrophy of the supraorbital bones (periorbital masses)
Periumbilical mass palpable (unchanged)
Verified pericardial effusion and faint cardiac sounds
Examining
MRI of the brain:
Lesions in the pons, cerebellar peduncles, and hemispheres that are T1 hypointense and T2 hyperintense
X-rays and bone scans: Hypercaptation on Tc-99 scan and symmetric distal long bone sclerosis
Peripheral hyperintensity on T2 and hyperpointense on T1/T2 MRI
Features of the disease (from case and literature)
Histiocytosis of rare, non-Langerhans cells
Lesions in both symmetrical bones (distal extremities)
Regular involvement of the CNS, cardiovascular system, peri-orbital, retroperitoneum, and occasionally the lungs
Renal artery compression brought on by retroperitoneal fibrosis may result in hypertension or renal failure.
CNS involvement: pyramidal symptoms, ataxia, and diabetes insipidus (if pituitary)
Protocol
Therapy and Results
At the time of the case, there was no successful standard treatment.
Steroids: no discernible change
Targeted treatments like BRAF inhibitors and interferon-alpha that were developed subsequently were not utilized for this patient.
With significant involvement of the heart, brain, or kidneys, the prognosis is poor.
Case outcome: Rapid neurological impairment and hypertension from renal artery compression caused the patient to pass away within weeks.
Issue: Skin Reactions Caused by Radiation
Details: Radiation exposure frequently causes erythema, dry or wet desquamation, and an increased risk of skin infections.
Interventions in Nursing:
Check the irradiated skin frequently for any changes (infection, redness, or disintegration).
Patients should be taught not to rub or scratch the area, to keep it dry and clean, and to stay away from irritants.
As directed, apply topical medications (such as steroid creams or hydrogels).
Keep an eye out for infection symptoms and notify the healthcare provider right away.
Issue: Fatigue Specifics: Cancer-related fatigue is common and impairs both cognitive and physical abilities.
Interventions in Nursing:
Use proven scales to systematically measure fatigue levels.
Promote frequent, mild exercise that the patient may tolerate, such as quick walks.
Schedule activities and care around times when you have more energy.
Teach patients and their families how to assign responsibilities and set priorities.
If necessary, assist in making referrals to support services.
Issue: Oral mucositis, particularly in radiotherapy to the head and neck
Details: The oral mucosa's painful inflammation and ulceration reduces nourishment and raises the risk of infection.
Interventions in Nursing:
Encourage good dental hygiene and conduct routine oral examinations.
Encourage the use of simple rinses (baking soda, salt) and steer clear of irritating or alcohol-based products.
Encourage the consumption of soft, non-irritating foods and enough water.
As directed, apply topical or systemic analgesics to relieve discomfort.
In order to control secondary infections, check for them and work with the oncology team.
Issue: Vomiting and Nausea
Details: Typical adverse effects brought on by the radiation therapy as well as the underlying cancer.
Interventions in Nursing:
Use established instruments to evaluate for severity and triggers.
Before and during treatment sessions, give antiemetic drugs as directed.
Encourage dietary changes, such as eating smaller, more frequent meals and staying away from fatty or spicy foods. Keep an eye out for symptoms of electrolyte imbalance or dehydration.
Issue: Anxiety and Psychological Distress Specifics: Patients and their families frequently experience severe emotional and psychological suffering as a result of receiving a cancer diagnosis and undergoing treatment.
Interventions in Nursing:
Regularly evaluate psychological status using validated diagnostic instruments and interviews.
In addition to providing information about the course of therapy and any adverse effects, offer emotional support.
Encourage dialogue between the interdisciplinary team, patients, and their families.
When necessary, make recommendations for support groups, social work, or counseling.
Issue: Infection Risk (because of immunosuppression)
Details: Chemotherapy and radiation exposure weaken the immune system, raising the risk of infection.
Interventions in Nursing:
Teach patients how to properly wash their hands and recognize illness symptoms.
To spot infections early, keep an eye on your temperature and other vital signs.
Implement infection prevention protocols (e.g., isolation if necessary, limiting visitors).
Collaborate with medical personnel to ensure timely action at the earliest indication of infection.
Notes
For more visit: 10.12659/AJCR.892750