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Journal of the association of physicians of india • JANUARY 2014 • VOL. 62

its impact on health-related quality of life. Clin Infect Dis 2004;39:342- 7. Andrew C. Nicholson, David P. Hajjar. Herpes viruses and thrombosis:
348. Activation of coagulation on the endothelium. Clinical Chimica Acta
3. Bodensteiner JB,Hille MR,Riggs JE. Clinical features of vascular 1999;286:23–29
thrombosis following varicella. Am J Dis Child 1992;146:100-2. 8. Minick CR, Fabricant CG, Fabricant J, Litrenta MM. Atheroarteriosclerosis
5. Steiner I, Kennedy PG, Pachner AR. “The neurotropic herpes viruses: induced by infection with a herpesvirus. Am J Pathol 1979;96:673-706
herpes simplex and varicella-zoster”. Lancet Neurol 2007;6:1015–28. 9. Ali MS, Kutty MS, Corea JR, Kutty K. Iliofemoral thrombosis in a case
6. Gogos CA, Apostolidou E, Bassaris HP, Vagenakis AG. Three cases of of chicken pox. Angiography 1984;35:577-8.
varicella thrombophlebitis as a complication of varicella zoster virus 10. Bodensteiner JB,Hille MR,Riggs JE. Clinical features of vascular
infection. Eur J Clin Microbiol Infect Dis 1993;12:43-5. thrombosis following varicella. Am J Dis Child 1992;146:100-2.

Immune Thrombocytopenia (ITP) : A Rare


Association of Lymphnode Tuberculosis
Anuj P Surana*, Kishor M Shelgikar**, Sameer Melinkeri***, Arvind Phadke****

Abstract
Although various haematologic abnormalities are known to occur with tuberculosis, association of immune
thrombocytopenia with tuberculosis is uncommon. We report a case of retroperitoneal lymph node
tuberculosis who presented with ITP. A 76 year old female was admitted to our hospital with oral mucosal
bleed and petechial lesions over extremities and abdomen. A diagnosis of immune thrombocytopenia
(ITP) was established. Intravenous Anti-D immunoglobulin and Dexamethasone therapy was started, but
failed to elicit any sustained platelet response. CT abdomen revealed multiple retroperitoneal lymph
nodes with central necrosis. Histopathology (HPE) of these revealed caseating lymphadenitis suggestive
of tuberculosis. After 2 months of anti-tuberculous therapy, the platelet counts returned to normal and
patient was off all therapy for ITP thereby suggesting likely association between tuberculosis and immune
thrombocytopenia.

Introduction

T uberculosis is one of the commonest infectious diseases in India. Various haematologic


abnormalities such as anaemia, leucocytosis, monocytosis, lymphopenia, leucopenia,
thrombocytopenia, thrombocytosis, leukemoid reactions and pancytopenia have been
described in tuberculosis 1 but immune thrombocytopenia (ITP) as the only presenting
feature of tuberculosis is extremely rare with few published reports. 2,3

*
Post Graduate Trainee, Case Report
Consultant Physician and
**

Diabetologist, ***Consultant A 76 year old female, known long standing diabetic and hypertensive on regular
Haematologist, ****Professor treatment, was admitted to our hospital with haemorrhagic bullae in oral cavity and
and Head of Department,
extensive petechial lesions over lower limbs and abdomen. There was no history of any
Department of Medicine,
Maharashtra Medical
haematological disorder or liver disease or significant history of any medication in the
Foundation, Joshi Hospital, 778, past apart from her usual anti-diabetic and anti-hypertensive medications.
Shivaji Nagar,Opposite Kamla On physical examination, patient was euthermic, pulse 80/min regular, blood pressure
Nehru Park, Pune 411 004.
160/90 mmHg. There was no palpable lymphadenopathy or any palpable organomegaly.
Received: 15.06.2012;
Revised: 13.08.2012; The initial total leucocyte count was 6890/cmm with differential count as follows:
Re-revised: 28.09.2012; neutrophils-57%, lymphocytes 33.5%, monocytes 7.5%, eosinophils 1.9%, basophils
Accepted: 09.02.2013

74  © JAPI • january 2014 • VOL. 62


Journal of the association of physicians of india • JANUARY 2014 • VOL. 62 75

Fig. 1 and 2 : CT scan demonstrating enlarged para-aortic, aorto-caval and retrocaval lymph nodes

0.1%. Haemoglobin was 12.1 g/dl; Platelet count on Two months following discharge, patient was seen
presentation was 1000 per cubic millimeter. Peripheral in good health with a platelet count of 3.4 lakhs / cmm.
smear examination was unremarkable apart from She had no side effects related to anti-tuberculous
paucity of platelets. Liver function tests, renal therapy. She is under regular follow up without any
function tests and serum electrolyte concentrations relapse of thrombocytopenia.
were normal and chest X-ray revealed no abnormality.
Prothrombin time (PT) and activated partial
Discussion
thromboplastin time (aPTT) were within normal limits ITP is an acquired disorder in which there is
and direct Coombs test was negative. Iron studies immune mediated destruction of platelets and possibly
revealed serum ferritin of 120 ng/ml and lipid profile inhibition of platelet release from megakaryocytes.
showed triglycerides of 74 mg/dl. ITP in children usually presents acutely while in
Subsequently, ultrasonography and CT scan of adults it runs a more chronic course, although at times
the abdomen (Figures 1 and 2) were done which it can have a very abrupt and stormy presentation.
demonstrated enlarged para-aortic, aorto-caval Common secondary causes of ITP include auto
and retrocaval lymphnodes - most showing central immune diseases, infections (HIV/hepatitis C) and
necrosis. Largest lymph node measured 2.6 x 1.7 drugs (rifampicin). 4
cm. Bone marrow aspiration and biopsy showed In the present case scenario, following possibilities
normocellular marrow with adequate megakaryocytes were considered in differential diagnosis:
consistent with peripheral platelet destruction. There
1. Disseminated Intravascular Coagulopathy: DIC
was no evidence of lymphoma or tuberculosis on bone
is a complex thrombo haemorrhagic disorder
marrow biopsy.
involving fibrinolysis and consumption of
During the course of her illness, the patient procoagulants and platelets. Typically, prolonged
received 6 units of random donor platelets (RDP’s). coagulation times, thrombocytopenia, high levels
There was only minimal and transient platelet of fibrin split products and microangiopathic
response to combined modality therapy with anti-rh 0 pathology (schistocytes) on peripheral smears are
immunoglobulin and high dose Dexamethasone. The suggestive findings. In our patient PT and aPTT
patient was started on Eltrombopag (Thrombopoietin were normal, thus ruling out DIC.
receptor agonist) 50 mg OD. Eight days of therapy
2. Thrombotic Thrombocytopenic Purpura (TTP):
effectively raised the platelet count to 9 lakhs / cmm.
Patients with TTP typically report an acute or
Eltrombopag was then withdrawn.
subacute onset of symptoms related to neurological
Subsequently an exploratory laparotomy was dysfunction, anaemia or thrombocytopenia.
performed for biopsy of retroperitoneal lymph F e ve r o c c u r s i n a p p r o x i m a t e l y 5 0 % o f t h e
nodes. Histo-pathological examination of these patients. Peripheral smears reveal moderate
lymph nodes revealed wide areas of caseation and to severe schistocytosis. Thus, TTP was ruled
scattered granulomas-indicating caseating tubercular out in our patient as there was no evidence of
l y m p h a d e n i t i s . A n t i - t u b e r c u l o u s t h e r a p y wa s microangiopathic haemolysis.
instituted with Isoniazid (300 mg) Ofloxacin (400 mg)
3. E va n s S y n d r o m e : E va n s s y n d r o m e i s t h e
Ethambutol (600 mg) and Pyrazinamide (1000 mg) and
co-existence of sequential or simultaneous
patient was discharged.
direct Coombs – positive AIHA (Autoimmune

© JAPI • january 2014 • VOL. 62  75


76 Journal of the association of physicians of india • JANUARY 2014 • VOL. 62

haemolytic anaemia) in conjunction with immune threatening situations.


mediated thrombocytopenia, with no known Our patient did not show any evidence of
underlying aetiology. In our patient Evans marrow infiltration with tuberculosis, neither
syndrome was ruled out as haemoglobin level was she exposed to anti-tubercular drugs prior to
was normal and direct Coombs test was negative. development of thrombocytopenia. Thus the only
4. Haemophagocytic Syndrome: It represents plausible explanation is immune mediated platelet
a severe hyperinflammatory condition with destruction associated with tuberculosis. This in itself
cardinal symptoms of prolonged fever, cytopenias, is exceedingly rare. 7 Anti-tubercular antibodies cross
hepato-splenomegaly and haemophagocytosis. In reacting with platelets can be a possible hypothesis
addition to pancytopenia, hypofibrinogenaemia for the same.
and hypertriglyceridaemia are also present, with It is interesting to note that our patient had
serum ferritin levels paralleling the course of a fulminant form of steroid and anti-D resistant
the disease. Hence in absence of pancytopenia ITP, necessitating a TPO agonist (Eltrombopag)
and any palpable organomegaly and presence t o a c h i e ve r e s p o n s e . E l t r o m b o p a g i s a s m a l l
of normal serum ferritin and triglyceride levels molecule Thrombopoietin receptor agonist for oral
Haemophagocytic syndrome was ruled out. administration. It interacts with the transmembrane
We t h e r e f o r e d e c i d e d t o i n v e s t i g a t e h e r domain of Thrombopoietin receptor (also known as
further with a CT scan of abdomen. It revealed C-MPI) leading to increased platelet production. 8,9
retroperitoneal lymphadenopathy with central However, treatment of tuberculosis itself led to
necrosis. Histopathological study showed caseation complete remission of ITP suggesting some possible
suggestive of tuberculosis. In absence of any other association.
convincing cause for severe thrombocytopenia, we ITP and tuberculosis are common diseases in India.
postulate a possible association of tuberculosis with A high index of suspicion of tuberculosis may be
her clinical presentation; although it may not be warranted in patients presenting with ITP as noted
directly causal. in our patient, the ITP may be cured with treatment
Pa t i e n t s w i t h b o t h p u l m o n a r y a n d e x t r a - of tuberculosis.
pulmonary tuberculosis may demonstrate
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