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CASE REPORT
Year : 2010  |  Volume : 6  |  Issue : 3  |  Page : 376-378

Spinal cord compression caused by anaplastic large cell lymphoma in an HIV infected individual


1 Department of Internal Medicine, Post Gradute Institute of Medical Education and Research, Chandigarh 160012, India
2 Department of Neurosurgery, Post Gradute Institute of Medical Education and Research, Chandigarh 160012, India
3 Department of Histopathology, Post Gradute Institute of Medical Education and Research, Chandigarh 160012, India
4 Department of Radiodiagnosis, Post Gradute Institute of Medical Education and Research, Chandigarh 160012, India

Date of Web Publication29-Nov-2010

Correspondence Address:
Susheel Kumar
Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh - 160 012
India
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DOI: 10.4103/0973-1482.73358

PMID: 21119283

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 > Abstract 

Lymphomas occur with an increased frequency in patients with Human Immunodeficiency Virus (HIV) infection. These are usually high-grade immunoblastic lymphomas and primary central nervous system lymphomas. Anaplastic large cell lymphoma (ALCL) is a distinct type of non-Hodgkin's lymphoma. It is uncommon in HIV infected individuals. We describe here an uncommon presentation of this relatively rare lymphoma in the form of spinal cord compression syndrome in a young HIV infected individual.

Keywords: Anaplastic large cell lymphoma, HIV infection, spinal cord compression


How to cite this article:
Kumar S, Wanchu A, Sharma A, Mukherjee K, Radotra B D, Gupta V, Singh S. Spinal cord compression caused by anaplastic large cell lymphoma in an HIV infected individual. J Can Res Ther 2010;6:376-8

How to cite this URL:
Kumar S, Wanchu A, Sharma A, Mukherjee K, Radotra B D, Gupta V, Singh S. Spinal cord compression caused by anaplastic large cell lymphoma in an HIV infected individual. J Can Res Ther [serial online] 2010 [cited 2014 Dec 19];6:376-8. Available from: http://www.cancerjournal.net/text.asp?2010/6/3/376/73358


 > Introduction Top


Acquired Immune Deficiency Syndrome (AIDS) related lymphoma (ARL) is usually a late manifestation of infection by the Human Immunodeficiency Virus (HIV), with a predilection for widespread, extra nodal disease, involvement of the central nervous system, and poor prognosis. The risk of non-Hodgkin's lymphoma (NHL) is about 150-250 fold higher among individuals with HIV infection compared with the general population. Anaplastic large cell lymphoma (ALCL) is a distinct type of NHL characterized by cohesive proliferation of large pleomorphic cells expressing CD 30. It is uncommon in HIV infected individuals. Herein we report a young HIV infected individual with ALCL who presented with features of spinal cord compression syndrome.


 > Case Report Top


A 16-years-old male, diagnosed to be HIV infected in 2002, presented to us with low-grade fever for six months and low backache for 15 days duration. Since 2002 he had been receiving antiretrovirals (Zidovudine+Lamivudine+Efavirenz) and was symptom free with CD4 count of 396 cells/mm 3 in august 2007. Fifteen days before current admission, he felt pain in lower dorsal and lumbar region. He developed sudden weakness along with decreased sensation in both lower limbs a week later, which progressed to complete flaccid paraplegia over the next three days. He was thinly built, with pallor and had generalized lymphadenopathy. He had local tenderness in the lower dorsal and lumbar region. Neurological examination revealed features of acute flaccid paraplegia with diminished sensation below L1 level. Other systems were unremarkable. Investigation revealed anemia (Hb=7.3 g/dl). Total and differential white cell and platelet counts were normal initially. He had hypo-albumenemia. ECG, Chest and dorso-lumbar spine X-ray and echocardiography were normal. Serum LDH level was within normal limits. Magnetic Resonance Imaging (MRI) of dorso-lumbar spine showed heterogeneously enhancing vertebral bodies at various levels of dorso-lumbar spine and extradural soft tissue from T9-L1 level causing cord compression [Figure 1]. As patient had documented rise in CD 4 count after initiation of antiretroviral therapy which supported a probability of longer life expectancy and there was possibility of other etiology besides lymphoma causing spinal cord compression, it was decided to take up the patient for surgery. The patient underwent T9-L1 laminectomy along with complete excision of extradural mass lesion. Pathological examination of mass lesion showed infiltration by markedly atypical cells, having hyperchromatic nuclei, prominent nucleoli. Immunohistochemistry for CD 30 showed strong Golgi zone positivity. CD 15 and CD 20 were negative. These features were consistent with a diagnosis of ALCL [Figure 2]. Bone marrow aspiration and biopsy showed infiltration by malignant cells with extensive myelofibrosis. Contrast-enhanced CT (CECT) of chest and abdomen showed enlarged retroperitoneal lymph nodes, hepato-splenomegaly, renomegaly and several hypodense lesions in liver.
Figure 1: MRI of spine (a) showing in saggital T2 and T1W images extradural soft tissue from T9 to L1 vertebral levels causing cord compression; (b,c) axial T1- and T2WI showing anterior displacement and compression of the dorsal cord by extradural soft tissue (arrows) which is extending along both the neural foramina; (d) axial T1 weighted post contrast image showing heterogenous enhancement of the extradural soft tissue

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Figure 2: Photomicrographs showing (a) many large atypical cells in a fibrous background admixed with lymphocytes (H&E ×40), (b) Immunostaining showing strong positivity for CD 30 in large atypical cells (Immunoperoxidase, ×40) and (c). Immunostaining showing occasional CD 20 positivity in lymphoid cells but atypical large cells are negative for CD 20 (Immunoperoxidase, ×40)

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Patient was given first cycle of cyclophosphamide, doxorubicin, vincristine and prednisolone (CHOP) regime and after stitch removal was planned for radio-therapy as well. Post surgery there was no improvement in neurological status. No complications related to sugery were noted during one month duration after surgery till his demise when he had a sudden cardiac arrest preceded by few episodes of vomiting.


 > Discussion Top


ALCL was first described as a clinical entity in 1985 by Stein et al. based upon cohesive proliferation of large pleomorphic cell's expressing CD30 (Ki-30). [1] It is a rare disease, accounting for less than 5% of all cases of NHL. [2] Presence of ALK protein is one of the most important prognostic factors. Five year survival is much better in patients with ALK+ ALCL as compared to ALK- ALCL. [3] ALCL is distinctly uncommon in HIV infected patients. Chadburn et al. described first ever case series of ALCL in HIV infected individuals. [4] To our knowledge, this is the first report of ALCL presenting as spinal cord compression in a HIV infected individual.

NHL involves spine in two ways. One is primary involvement of only spinal epidural space with no other systemic involvement. This entity is known as primary spinal epidural NHL. Second is involvement of epidural space in a setting of disseminated NHL. NHL has spinal localization in 0.1-6.5% of cases. [5] NHL presenting for first time as spinal cord compression is rare and occurs in less than 5% of cases. This type of presentation in HIV infected patients is as case reports only. Petit et al. reported 13 cases of NHL presenting as spinal cord compression. One patient had AIDS associated Burkitts' lymphoma. [6]

Treatment in patients with diagnosed cases of lymphoma presenting with features of spinal cord compression is generally spinal irradiation and chemotherapy. Patients with symptomatic spinal cord compression and unknown lesions require surgical decompression for diagnosis as well as treatment. CHOP is a standard chemotherapeutic regime used for the treatment of high grade lymphoma including ALCL. [7] A recent study found 66% of untreated AIDS-related lymphomas have multidrug resistance 1 (MDR-1), which correlated with poor clinical outcome. [8] Infusion schedules can overcome MDR-1 in vitro, suggesting that dose-adjusted EPOCH (etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin) or other infusional regimens like CDE (cyclophosphamide, doxorubicin, and etoposide) may be particularly beneficial in ARL. Little et al. achieved a complete remission in 79% of patients along-with 92% disease free survival at 53 months median follow-up in patients with AIDS-related lymphoma with dose-adjusted EPOCH infusional regimen. [9] Nagajothe et al. reported 2 cases of ALCL in HIV Positive patients who showed excellent response with dose-adjusted EPOCH. [10]

This case report describes an uncommon presentation of this relatively rare lymphoma in the form of spinal cord compression syndrome in a young HIV infected individual.

 
 > References Top

1.Stein H, Mason DY, Gerdes J, O'Connor N, Wainscoat J, Pallesen G, et al. The expression of the Hodgkin's disease associated antigen Ki-1 in reactive and neoplastic lymphoid tissue: Evidence that Reed-Sternberg cells and histiocytic malignancies are derived from activated lymphoid cells. Blood 1985;66:848-58.  Back to cited text no. 1
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2.Kadin ME, Morris SW. The t(2;5) in human lymphomas. Leuk Lymphoma 1998;29:249-56.  Back to cited text no. 2
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3.Gascoyne RD, Aoun P, Wu D, Chhanabhai M, Skinnider BF, Greiner TC, et al. none Prognostic significance of anaplastic lymphoma kinase (ALK) protein expression in adults with anaplastic large cell lymphoma. Blood 1999;93:3913-21.  Back to cited text no. 3
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4.Chadburn A, Cesarman E, Jagirdar J, Subar M, Mir RN, Knowles DM. none CD30 (Ki-1) positive anaplastic large cell lymphomas in individuals infected with the human immunodeficiency virus. Cancer 1993;72:3078-90.  Back to cited text no. 4
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5.Salvati M, Cervoni L, Artico M, Raco A, Ciappetta P, Delfini R.none Primary spinal epidural non-Hodgkin's lymphomas: A clinical study. Surg Neurol 1996;46:339-43.  Back to cited text no. 5
[PUBMED]  [FULLTEXT]  
6.Petit B, Roche I, Paraf F, Jaccard A, Boneoeur MP, Moreau JJ, et al. Epidural non-Hodgkin's lymphoma presenting as a spinal cord compression. Ann Pathol 2001;21:123-8.  Back to cited text no. 6
    
7.Fisher RI, Gaynor ER, Dahlberg S, Oken MM, Grogan TM, Mize EM, et al. Comparison of a standard regimen (CHOP) with three intensive chemotherapy regimens for advanced non-Hodgkin's lymphoma. N Engl J Med 1993;328:1002-6.  Back to cited text no. 7
[PUBMED]  [FULLTEXT]  
8.Tulpule A, Sherrod A, Dharmapala D, Young LL, Espina BM, Sanchez MN, et al. Multi-drug resistance (MDR-1) expression in AIDS-related lymphomas. Leuk Res 2002;26:121-7.  Back to cited text no. 8
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9.Little RF, Pittaluga S, Grant N, Steinberg SM, Kavlick MF, Mitsuya H, et al. Highly effective treatment of acquired immunodeficiency syndrome-related lymphoma with dose-adjusted EPOCH: Impact of antiretroviral therapy suspension and tumor biology. Blood 2003;101:4653-9.  Back to cited text no. 9
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10.Nagajothi N, Dham SK, Gelfand Y, Sanmugarajah J. Treatment of AIDS-associated anaplastic large-cell lymphoma with dose-adjusted EPOCH chemotherapy. J Natl Med Assoc 2007;99:799-801.  Back to cited text no. 10
[PUBMED]  [FULLTEXT]  


    Figures

  [Figure 1], [Figure 2]


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