The natural artistry of disease: a wintry landscape in the eye

November 09, 2020

Researchers from Tokyo Medical and Dental University (TMDU) report a case of frosted branch angiitis in a woman presenting years after being treated for leukemia-lymphoma with allogeneic human stem cell transplant.

Tokyo, Japan - Leukemias and lymphomas are life-threatening malignancies affecting white blood cells and the immune system; fortunately, radiotherapy and chemotherapy, combined with stem cell therapy, can improve survival significantly. Now, Japanese researchers report one such patient who presented with an intriguing and rare delayed ocular complication descriptively named frosted branch angiitis.

A Japanese woman in her early fifties was diagnosed with acute-type adult T-cell leukemia-lymphoma associated with human T-cell lymphotropic virus type 1 (HTLV-1). She was managed with whole body irradiation and anti-cancer drugs, followed by allogeneic (donor) human stem cell transplant (HSCT). HSCT allows physicians to use higher doses of chemoradiotherapy as the patient gets an infusion (transplant) of blood-forming stem cells to restore collaterally damaged bone marrow. The patient went into remission and had no complications for several years.

Over four years after HSCT, she developed blurred vision and was referred for further examination. A slit-lamp biomicroscope, which shines a thin sheet of light into the eye for inspection, revealed cellular infiltrates in both segments. Associate Professor Koju Kamoi, lead / corresponding author, describes the remarkably picturesque fundoscopy findings. "Along the retinal blood vessels, a translucent sheath was visible resembling the icy branches of a tree in winter. We recognized this as frosted branch angiitis (Figure A), a rare presentation of florid retinal vasculitis."

The medical team ran a battery of tests to rule out recurrence, other autoimmune disease, and bacterial, viral or fungal infections. Therefore, the physicians concluded that the frosted branch angiitis resulted from immune activation following HSCT. First described in the Japanese literature by Ito in 1976 occurring in a six-year-old child, frosted branch angiitis is very rare, with most cases reported from Japan and sporadically from other countries.

The patient was treated with corticosteroids to suppress inflammation. After six months the lesion regressed but steroids were continued for over a year. Surprisingly, a month after stopping systemic steroids, the patient developed a skin rash (Figure B) and dryness of both eyes. "This sequence of events suggests that frosted branch angiitis was likely the first sign of systemic inflammation," explains Dr Kamoi. "Steroids could have suppressed the slower skin and eye-surface changes that were expressed following discontinuation."

"Though HSCT is beneficial in many situations, it may activate the immune system adversely," warns Professor Kyoko Ohno-Matsui, senior author. "Frosted branch angiitis could serve as early warning sign of inflammation elsewhere in the body. Therefore, intraocular monitoring is warranted in these patients."
The article, "Frosted branch angiitis after allogeneic haematopoietic stem cell transplantation in adult T-cell leukaemia-lymphoma" was published in Lancet Haematology. at DOI: 10.1016/S2352-3026(20)30226-X

Tokyo Medical and Dental University

Related Immune System Articles from Brightsurf:

How the immune system remembers viruses
For a person to acquire immunity to a disease, T cells must develop into memory cells after contact with the pathogen.

How does the immune system develop in the first days of life?
Researchers highlight the anti-inflammatory response taking place after birth and designed to shield the newborn from infection.

Memory training for the immune system
The immune system will memorize the pathogen after an infection and can therefore react promptly after reinfection with the same pathogen.

Immune system may have another job -- combatting depression
An inflammatory autoimmune response within the central nervous system similar to one linked to neurodegenerative diseases such as multiple sclerosis (MS) has also been found in the spinal fluid of healthy people, according to a new Yale-led study comparing immune system cells in the spinal fluid of MS patients and healthy subjects.

COVID-19: Immune system derails
Contrary to what has been generally assumed so far, a severe course of COVID-19 does not solely result in a strong immune reaction - rather, the immune response is caught in a continuous loop of activation and inhibition.

Immune cell steroids help tumours suppress the immune system, offering new drug targets
Tumours found to evade the immune system by telling immune cells to produce immunosuppressive steroids.

Immune system -- Knocked off balance
Instead of protecting us, the immune system can sometimes go awry, as in the case of autoimmune diseases and allergies.

Too much salt weakens the immune system
A high-salt diet is not only bad for one's blood pressure, but also for the immune system.

Parkinson's and the immune system
Mutations in the Parkin gene are a common cause of hereditary forms of Parkinson's disease.

How an immune system regulator shifts the balance of immune cells
Researchers have provided new insight on the role of cyclic AMP (cAMP) in regulating the immune response.

Read More: Immune System News and Immune System Current Events is a participant in the Amazon Services LLC Associates Program, an affiliate advertising program designed to provide a means for sites to earn advertising fees by advertising and linking to