A Unique Case Of Transient Spontaneous Regression Complicated With Tumor Lysis Syndrome Of T-cell Lymphoblastic Lymphoma In Hiv-infected Patient Without Antiretroviral Therapy.
Rujirojindakul, P., Kayasut, K., Rohitoprakarn, M., & Lekhakula, A. (2007). A unique case of transient spontaneous regression complicated with tumor lysis syndrome of T-cell lymphoblastic lymphoma in HIV-infected patient without antiretroviral therapy. Journal of the Medical Association of Thailand = Chotmaihet thangphaet, 90 9, 1930-3 .
View Original Source →Abstract
Spontaneous regression in high-grade non-Hodgkin lymphoma is rare. Herein, the authors report the case of a 26-year-old asymptomatic HIV-infected patient presenting with bleeding per gum after a dental extraction. Initially, a complete blood count showed lymphoblasts and thrombocytopenia. Laboratory investigations were compatible with acute tumor lysis syndrome. Without any steroid or chemotherapy, both clinical and laboratory abnormalities were spontaneously returned to normal limits. However, three weeks later he developed generalized lymphadenopathy. A submandibular gland biopsy revealed to be T-cell lymphoblastic lymphoma. This was followed by the second episode of spontaneous tumor lysis syndrome and spontaneous regression of lymphadenopathy again. At this time, he was treated with cyclophosphamide, adriamycin, vincristine, and prednisolone (CHOP) with whole brain irradiation. During seven months of chemotherapy, the physical examination and blood chemistry were normal. Unfortunately, after seven courses of CHOP, the disease rapidly progressed and ultimately lead to his death.
Case Details
Disease Location
Submandibular gland lymph nodes
Personal Characteristics
26 -year-old male september 2001 medical history was unremarkable, but did have perform sexual acts with a prostitute years earlier, which was the only known risk factor for the patients HIV
Clinical Characteristics
Presented with bleeding in his gums for seven dayafter a dental extraction physical exam showed generalized petechiae and purpura without any lymphadenopathy or hepatosplenomegaly. Complete blood count showed normal hemoglobin, thrombocytopenia, leukocytosis, and 90% lymphoblasts. There was no immunophenotype confirmation during admission, he had received only platelet and packed red cell transfusion without steroid or chemotherapy. 4 days later he developed acute renal failure and pulmonary hemorrhage and was transferred to another hospital on admission to the other hospital, he was pale, febrile, tachycardic, and normotensive with endotracheal intubation. Cbc showed normal leukocyte count with leucoerythroblastic features (nucleated red blood cell 2%, myelocyte 2%, and metamyelocyte 2%), hb was 7.9gm/dl, and platelet count was 67x10^9/l. Bone marrow aspiration was non-diagnostic. Results from investigations of acute tumor lysis syndromee were: blood urea nitrogen (bun) 123.9mg/dl, creatinine (cr) 9.3mg/dl, potassium (k) 7.4mmol/l, bicarbonate (hco3) 17mmol/l, calcium (ca)7.2mg/dl and phosphate (p) 14.5mg/dl, uric acid 34mg/dl. Serum lactate dehydrogenase was elevated 1,036 u/l. HIV antibodies were positive. Ultrasonography of the urinary system revealed bilateral nephromegaly with a significatn increase of renal echogenicity and reserved corticomedullary differentiation that was compatible with infiltrative parenchymal disease. Microbiologic studies for bacteria and fungus were negative. Ventilator supporight was introduced with platelet and prc replacement three weeks, physical exam revealed bilateral cervical, submandiubular and preauricular lymphadenopathy but showed improvements (see remission characteristics) one week later, the patient was readmitted because of developing tonic-clonic seizure. He still had generalized lymphadenopathy apart from papilledema, no neurological deficit was detected CT scan of the brain without contrast showed multiple hypodense lesions cbc was normal but acute tumor lysis syndrome was detected. Lab results were bun 36.5mg/dl, cr 4.49mg/dl, k 4.6mmol/l, hco3 16mmol/l, ca 10mg/dl, p 6.6mg/dl, uric acid 37.4mg/dl and serum ldh was high (2,062 u/l) a submandiubular land biopsy revealed lymphoblastic lymphoma immunohistochemistry indicated t-cell origin with staining for lca and cd45ro but not CD20. Bone marrow biopsy didnt reveal any malignancy after the 7th course of chop he was admitted again because of developing a severe headache and right lateral rectus palsy. Csf revealed numerous lymphoblasts. Systemic manifestation progressed rapidly which eventually led to death
Remission Characteristics
After ventilator supporight and platelet and prc replacement, the cbc and all the lab results returned to normal within 8 days three weeks later, although lymphadenopathies were revealed, cbc was normal and a ln biopsy showed a lymphoid depletion and negative acid-fast and fungus stain all enlarged ln spontaneously decreased in size after the biopsy and simultaneously the lab results were normal with only admin of IV saline and allopurinol after chop, all lymphadenopathy completely disappeared after whole brain radiation, no abnormalities were detected from CT images he had been asymptomatic and no evidence of disease was found for more than 6 months
Treatment & Mechanisms
Proposed Remission Mechanisms
Coincident events, infection, or biopsy may result in an augmented host repsonse that results in an immunologocially mediated tumor regression apoptosis, angiogenesis inhibitor, induction of differentiation, or hormonal effects
Clinical Treatment
Transfusion ventilator supporight with platelet and prc replacement IV saline and allopurinol chop regimen brain radiation
Non-Clinical Treatment
None reported