Rare case of PNH plus lymphoma highlights management challenges
People with acquired blood disorder more likely to develop blood cancers
A rare case of paroxysmal nocturnal hemoglobinuria (PNH) coexisting with diffuse large B-cell lymphoma (DLBCL), a type of blood cancer, was chronicled in a new report that sought to elucidate some of the challenges that can come with the management of the acquired blood disorder co-occurring with this cancer.
Data show that people with PNH, a condition in which the immune system mistakenly destroys red blood cells, also have an increased risk of developing certain blood cancers, among them acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS).
DLBCL is the most common type of non-Hodgkin lymphoma, a fast-growing, but treatable, blood cancer that starts in immune B-cells.
The researchers noted that “in contrast to the well-documented progression of PNH to MDS/AML, secondary lymphoma development is rarely reported.” Therefore, the team wrote, “the current case report may highlight the challenges and considerations for managing DLBCL coexisting with PNH and serve as a valuable reference for clinicians.”
Their study, “Diffuse Large B-Cell Lymphoma Combined With Paroxysmal Nocturnal Hemoglobinuria: A Rare Case Report,” was published in Clinical Case Reports.
People with PNH typically experience symptoms such as anemia, fatigue, dark urine, and blood clots. DLBCL, meanwhile, usually presents as a rapidly growing, painless mass, most commonly in the lymph nodes of the neck, armpit, or groin.
Because the coexistence of PNH and DLBCL is rare, the researchers decided to detail the case of a woman with the blood disorder who was subsequently diagnosed with the blood cancer. The woman, 41, had a three-year history of hemolytic anemia, or anemia caused by the destruction of red blood cells, and had been treated intermittently with steroids and blood transfusions.
Over the previous year, however, she had developed progressive right-leg pain, weakness, and impaired mobility, along with a weight loss of 10 kg (22 lbs) in four months.
CT scans showed enlarged lymph nodes in several areas of woman’s body
CT scans obtained 10 days before admission showed multiple enlarged lymph nodes in several areas of her body, including around the collarbone, in the abdomen, and in the pelvic regions. Further tests demonstrated a substantial loss of GPI-anchored proteins on certain blood cells, the underlying cause of PNH, further confirming her PNH diagnosis.
On examination, she appeared pale and jaundiced, with yellowing of the skin and whites of the eyes. She also had enlarged lymph nodes in the neck and armpits, and marked weakness in the right lower leg.
A neck lymph node biopsy revealed diffuse large B-cell lymphoma with the non-germinal center B-cell (non-GCB) subtype, which is often associated with poorer outcomes than the GCB subtype. A bone marrow analysis did not detect B-cell proliferation, indicating the absence of detectable cancer in the bone marrow.
To control hemolysis, she received intravenous, or into-the-vein, methylprednisolone, a steroid, and sodium bicarbonate. She also was given transfusions of red blood cells and platelets, the cell fragments that help blood clot. Blood tests revealed a decrease in lactate dehydrogenase (LDH) levels, a marker of tissue damage. She was also given an antibiotic (ceftazidime) for bacterial lung infections.
Researchers cited need for comprehensive evaluation, close monitoring
The woman began chemotherapy with the R-CHOP regimen, a standard combination of five medicines used primarily to treat aggressive non-Hodgkin lymphoma. Even though there was some shrinkage of the lymph nodes, she experienced a severe drop in blood cell counts, known as myelosuppression, and severe pneumonia. A second course of R-CHOP was then administered.
The day after chemotherapy, her LDH levels rose sharply, indicating significant hemolysis, with CT scans of the chest showing worsening lung infection. Over the following week, she developed blood clots in both of her legs and more severe myelosuppression.
Despite treatment with intravenous sodium bicarbonate, dexamethasone, and heparin, her hemolysis remained uncontrolled, and she developed blood clots in the veins of her arms. Soon after, her condition rapidly deteriorated, and she ultimately died from respiratory failure, caused by combined lung infection and blood clots.
The researchers noted that it’s rare to see a report on “the occurrence of lymphoma after PNH treatment.” Further, “no research has yet identified specific gene mutations in PNH patients that correlate with an increased risk of lymphoma,” the team wrote.
Citing the “distinct clinical characteristics” of DLBCL coexisting with PNH, the researchers urged that patients with both conditions “be comprehensively evaluated before chemotherapy,” with “close monitoring” during treatment to quickly deal with any challenges that may arise.
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