Keywords
Hodgkin lymphoma - immune thrombocytopenia - thrombocytopenia
Introduction
Approximately 30% of secondary immune thrombocytopenia (ITP) cases are associated
with lymphoid tumors.[1] ITP in Hodgkin lymphoma (HL) is reported rarely accounting for only 0.2%–1% of all
the LDPs-ITP.[2] We report a case of HL presenting with thrombocytopenia at diagnosis.
Case Report
A 12-year-old girl presented with epistaxis, gum bleeding, and hematuria for 1 month.
She had no history of fever, joint pain, and diarrhea. Her menarche was not achieved.
She had received 3 units of platelet transfusion from outside hospital. On examination,
she had pallor. In the left cervical region multiple firm, mobile nontender nodes
were palpable largest measuring 4 cm × 3 cm. Systemic examination was unremarkable
(no organomegaly was observed). As per the patient's mother, the lymph nodes were
enlarged for the past 6 months; however, no investigations or treatment was done.
Her hemoglobin was 10 g/dl, white blood cell count was 11 × 109/L with predominance of neutrophils (75%), and platelet count was 20 × 109/L. No immature cells were observed in peripheral smear. Her lactate dehydrogenase
was raised to 459 IU/L. Other serological tests including liver function, renal function,
viral marker (Epstein–Barr virus [EBV], HIV, hepatitis B surface antigen, and hepatitis
C virus) and autoimmune markers were unremarkable. Her prothrombin time, activated
thromboplastin time was within the reference range. Fine-needle aspiration cytology
(FNAC) smears from cervical lymph node showed a fair number of mononuclear Hodgkin
cell and occasional binucleate and multinucleate Reed–Sternberg cell in a background
of lymphocytes, plasma cells, neutrophils, and eosinophil. In view of FNAC findings,
lymph node biopsy was done which confirmed HL of mixed cellularity type [Figure 1]. Staging work including imaging and bone marrow examination was done. Noncontrast
computerized tomography scan of neck, thorax, and abdomen revealed multiple level
II/III lymph nodes in the left cervical region largest measuring 5 cm × 2.5 cm. Also
noted were hepatosplenomegaly along with multiple enlarged lymph nodes in perigastric,
paracaval, aortocaval, and perisplenic region. Bone marrow examination did not show
infiltration by Reed–Sternberg cells. In addition, the megakaryocytes were increased,
in clusters, with predominance of early forms [compensatory response to thrombocytopenia,
[Figure 2].[3] A final diagnosis of HL mixed cellularity stage III B bulky disease with ITP was
made.
Figure 1: Cervical lymph node biopsy (H and E, ×400): multiple Reed–Sternberg cells
(arrow) along with background population of eosinophil, lymphocyte, and histiocytes
Figure 2: Bone marrow aspirate (Leishman stain × 200): Megakaryocyte cluster, with
increased early forms
The patient was given 6 cycles of adriamycin, bleomycin, vinblastine, and dacarbazine
(ABVD) followed by local radiation to the neck. Her platelet count further improved
to 240 × 109/L after the first cycle of ABVD. The patient is now in remission 18 months after
the end of treatment.
Discussion
Autoimmune disorders have been previously associated with HL.[4] In an analysis of 1029 patients of HL 3 had ITP.[5] ITP may occur before, concurrent with, at the time of recurrence of lymphoma or
in complete remission after treatment. In HL majority cases have been reported at
follow-up.[2]
[5] Few cases have presented with thrombocytopenia at diagnosis.[4]
[5]
[6]
[7] Our case presented with symptomatic thrombocytopenia at the diagnosis of HL. The
patients with cytopenia (thrombocytopenia or anemia) at diagnosis have been shown
to be older, with advanced disease and nonnodular sclerosis histology as compared
to patients without cytopenia at diagnosis.[5]
[8] Our case was stage III B with mixed cellularity histology in concordance with the
previous reports. ABVD combination therapy effectively controlled both HL and autoimmune
condition in our patient as in previous reports. Immunomodulation with steroid or
immunoglobulins has also been used in management.[7]
ITP and HL may be linked as a part of paraneoplastic disorder, an uncontrolled immune
response to the malignant cell proliferation or simultaneous but unrelated disease.
The immune destruction of platelets may result from IgG autoantibodies that coat platelet,
complement-mediated destruction of platelet by IgM, direct inhibition of megakaryocytopoiesis
by cytotoxic T cell and inhibitory cytokines. The points in favor of immune destruction
of platelets were increased proliferative megakaryocyte response and failure to respond
to platelet transfusion. In addition, the improvement of platelet count after the
first cycle of ABVD supports a causative association between the two.
Our case highlights the clinical heterogeneity of HL. The initiation of definitive
therapy for the lymphoma is the best way to restore the platelet counts.
Declaration of patient consent
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understand that their names and initials will not be published and due efforts will
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