Case Report
 
Antinuclear antibodies positive patient with splenic infarct a diagnostic dilemma
Sabina Langer1, Ravi Daswani2, Rahul Arora3, Nitin Gupta4, Anil Arora5, Jyoti Kotwal6
1Consultant, Department of Haematology, SirGangaram Hospital, New Delhi, Delhi, India.
2DNB Fellow, Department of Gasteroenterology, Sir Gangaram Hospital, New Delhi, Delhi, India.
3DNB Fellow, Departement of Haematology, Sir ganga Ram Hospital, New Delhi, India.
4Consultant, Clinical Haematology, Department of Haematology, Sir Gangaram Hospital, New Delhi, Delhi, India.
5Senior, Consultant, Department of Institute of Liver Gastroenterology & Pancreatico-Biliary Sciences, Sir Gangaram Hospital, New Delhi, Delhi, India.
6Senior, Consultant & Head, Department of Haematology, Sir Gangaram Hospital, New Delhi, Delhi, India.

Article ID: Z01201604CR10630SL
doi:10.5348/ijcri-201642-CR-10630

Address correspondence to:
Dr. Sabina Langer
Consultant, Department of Hematology
Sir ganga Ram Hospital
New Delhi, 110005

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How to cite this article
Langer S, Daswani R, Arora R, Gupta N, Arora A, Kotwal J. Antinuclear antibodies positive patient with splenic infarct a diagnostic dilemma. Int J Case Rep Imag 2016;7(4):245–248.


Abstract
Introduction: Presence of autoimmune phenomenon along with splenic infarct made clinician relate the two considering the splenomegaly as a consequence of splenic vein thrombosis and autoimmune myelofibrosis as a cause of pallor and repeated blood transfusions. The presence of near normal counts masked the underlying myeloproliferative neoplasm which led to the patient wrongly being treated as autoimmune myelofibrosis. This case highlights the need to keep a high level of suspicion for chronic myeloproliferative neoplasms in all cases of splanchnic vein thrombosis. The autoimmune phenomenon including Antinuclear antibodies (ANA) positivity is incidentally found to coexist with the primary myelofibrosis.
Case Report: A 69-year-old male presented with splenic infarct and ANA positivity. The splenomegaly was initially attributed to the splenic vein thrombosis and autoimmune phenomenon like Raynaud's phenomenon and skin rash bought clinician's attention. The presence of splenic vein thrombosis, near normal counts with mainly pallor the patient was been treated on the lines of autoimmune myelofibrosis. The bone marrow done pointed towards the diagnosis of primary myelofibrosis which was confirmed by molecular studies positive for JAK-2 mutation.
Conclusion: Autoimmune phenomenon can coexist with the chronic myeloproliferative neoplasms like primary myelofibrosis. All patients presenting with splanchnic vein thrombosis should be investigated for underlying myeloproliferative neoplasms especially molecular studies like JAK-2 V617F mutations.

Keywords: Primary myelofibrosis, Splenic infarct, Thrombosis


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Author Contributions
Sabina Langer – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Ravi Daswani – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Rahul Arora – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Nitin Gupta – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Anil Arora – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Jyoti Kotwal – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Guarantor of submission
The corresponding author is the guarantor of submission.
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None
Conflict of interest
Authors declare no conflict of interest.
Copyright
© 2016 Sabina Langer et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.