spontaneous regression of primary progressive Hodgkin's lymphoma in a pediatric patient: a case report and review of literature

Abstract

Spontaneous regression of malignancies is a very rare phenomenon. Our research of existing literature yielded only 16 cases of Hodgkin's lymphoma which regressed spontaneously. The outcome of primary progressive Hodgkin's lymphoma is poor even with salvage chemotherapy and autologous bone marrow transplantation. Here we present a case of primary progressive Hodgkin's lymphoma, which regressed spontaneously after failure of salvage chemotherapy. To our knowledge, this is the first case report of primary progressive Hodgkin's lymphoma undergoing spontaneous regression.

Case Details

Disease Location

Lymph nodes (hl)

Personal Characteristics

10 -year-old female history of fever and cough of two months malnourished height 118cm, weight 18kg, bmi 12.9kg/m^2

Clinical Characteristics

Presented in september 2009 she had right level 4 lymph nodes measuring 1x1cm and splenomegaly of 3cm below left costal margin chest x-ray showed mediastinal widening cervical lymph node biopsy was diagnostic of hodgkin's lymphoma of the nodular sclerosis type, which was latent membrane protein 1, CD15 and CD30 positive contrast CT chest revealed multiple mediastinal lymphadenopathies with the largest right paratracheal ln measuring 3.7x3.6cm. Contrast CT of abdomen revealed retroperitoneal lymph nodes with mild splenomegaly the patient's hemoglobin 70g/l, total white cells 8.7x10^9/l (lymphocytes 20%, neutrophils 75%, monocytes 4%, and basophils 1%), platelet count of 399x10^9/l, total serum protein was 61g/l with albumin of 36g/l, and lactate dehydrogenase was 1120u/l. Diagnosed with hl, nodular sclerosis stage 3b and was planned for six to eight cycles of chemo started chemo in oct 2009 and complete 8 cycles in may 2010. At the end of the treatment, she had bilateral axillary lymph nodes of 1x1cm and bilateral inguinal lymph nodes around 1.5x1cm PET revealed increased uptake in above mentioned nodes and also sclerotic lytic lesions in d5, 6, 9, l1 and l5 vertebra. There were lytic lesions in the pelvic bone involving bilateral ilium, left acetabulum and the left femur involving the intertrochanteric region bone marrow biopsy showed small area of fibrosis along with numerous r-s cells which were CD15 and CD30, suggesting a positive involvement of bone marrow. Diagnosed with primary progressive hl she then received 4 cycles of salvage chemotherapy-dhap from august 2010 to october 2010 after the salvage chemo, she had subcentimetric axillary lymph nodes and inguinal lymph nodes PET showed decreased uptake of above mentioned nodes compared to that performed in may 2010, but showed new areas of uptake with a well-defined hypodense lesion in segment 7 of the liver measuring 1.1x1cm, nodes involving prevertebral and retrocaval regions and persisting bone lesions repeat bone marrow biopsy showed lymphomatous involvement patient wanted supportive care only and was discharged in january 2011

Remission Characteristics

14 months later from discharge, in march 2012 the patient was absolutely asymptomatic with weight of 25kg and height of 125cm, bmi of 16. There was report of no chemotherapy agents used or alternative medicines on exam, she only had subcentimetric bilateral inguinal lymph nodes PET repeated showed complete revolution of the hepatic lesion and all bone lesions nodes in the bilateral inguinal region and in the prevertebral and retrocaval regions did not show any metabolic uptake bone marrow biopsy was normal in the last follow-up in january 2013, she is clinically free of lymphadenopathy

Treatment & Mechanisms

Proposed Remission Mechanisms

Common mechanisms proposed include immunologic factors, elimination of carcinogens, concomitant infections, hormonal factors, genetic factors, surgical trauma, and induction of differentiation. Immunological is most commonly attributed to sr no major mechanism proposed for this exact case

Clinical Treatment

Started copp/abv: cyclophosphamide, vincristine, procarbazine, prednisone, doxorubin, bleomycin, and vinblastine in oct 2009 4 cycles of slave chemo-dhap (dexamethasone, high dose cytarabine, and cisplatin)

Non-Clinical Treatment

None reported