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Year : 2015  |  Volume : 6  |  Issue : 2  |  Page : 85-87

Thrombocytopenia and hemorrhagic pleural effusion as an initial presentation of polycythemia vera

Department of Medicine, Mahatma Gandhi Institute of Medical Sciences, Sevagram, Wardha, Maharashtra, India

Date of Web Publication7-Jul-2015

Correspondence Address:
Amrish Saxena
Department of Medicine, MLK 5, MGIMS Campus, Sevagram, Wardha, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1658-5127.160210

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Polycythemia vera (PV) is a chronic myeloproliferative disorder in which there is an alteration in the pluripotent progenitor cell leading to excessive clonal proliferation of erythroid, myeloid and megakaryocytic progenitor cells. The natural history of PV can be divided into several stages, beginning with asymptomatic, isolated erythrocytosis, progressing to more generalized myeloid proliferation, splenomegaly, and thrombosis, followed by myelofibrosis, leukoerythroblastosis, cytopenia, and myeloid metaplasia and sometimes, acute leukemia. Isolated erythrocytosis, leukocytosis, or thrombocytosis or in combination are usually present at the onset of disease. We present the case of a 65-year-old man, who developed thrombocytopenia and hemorrhagic pleural effusion as an initial presentation of PV that is extremely rare.

Keywords: Complications, hepatosplenomegaly, pleural effusion, polycythemia vera, thrombocytopenia

How to cite this article:
Saxena A, Bodkhe S, Mishra D, Singh V. Thrombocytopenia and hemorrhagic pleural effusion as an initial presentation of polycythemia vera. J Appl Hematol 2015;6:85-7

How to cite this URL:
Saxena A, Bodkhe S, Mishra D, Singh V. Thrombocytopenia and hemorrhagic pleural effusion as an initial presentation of polycythemia vera. J Appl Hematol [serial online] 2015 [cited 2023 Oct 2];6:85-7. Available from: https://www.jahjournal.org/text.asp?2015/6/2/85/160210

  Introductio Top

Polycythemia vera (PV) is a clonal hematopoetic stem cell disorder with trilineage myeloid involvement. [1] It is characterized by growth factor-independent erythroid proliferation producing an elevated red cell mass. The incidence of PV is 2/100,000 persons. Thrombotic events (coronary events, cerebrovascular accidents, deep vein thrombosis, pulmonary embolism, mesenteric, hepatic, portal, or splenic vein thrombosis) are a major complication of PV. [2],[3] A cerebrovascular event precedes the diagnosis in 35% of patients with PV. [1] Both bleeding and thrombosis can occur in PV. [4]

Over the last two decades, hemostatic abnormalities in myeloproliferative neoplasms have been the subject of extensive investigation aimed at elucidating their pathogenesis and clinical manifestations, toward providing effective and safe treatment options. Significant progress has been made in the understanding of clinical epidemiology and pathogenesis of thrombohemorrhagic manifestations, particularly in patients with PV and essential thrombocythemia (ET). [3]

Hemorrhagic diathesis is more rare than thrombotic events in PV and ET and mostly affects patients with a very high platelet count. In these patients, an altered degradation and function of von Willebrand factor or qualitative platelet defects can cause mucocutaneous hemorrhages, which may lead to major gastrointestinal bleedings. [4] The bleeding tendency can be effectively treated by cytoreduction. [1],[4]

  Case report Top

A 65-year-old man was admitted with a history of generalized weakness, easy fatigability, weight loss and pruritus for 2 months. He denied any history of fever, cough, breathlessness, headache, visual disturbances, vertigo, tinnitus, hypertension, cerebrovascular stroke, or myocardial infarction. He had a 30 pack-year smoking history and 2 years history of diabetes mellitus. On physical examination, he was conscious, oriented, afebrile, anicteric with stable vitals. His tongue was smooth, shiny and red [Figure 1]. Multiple erythematous excoriated papules with scratch marks were present all over the body especially over the trunk [Figure 2]. Petechiae, ecchymosis, mucosal bleeding and lymphadenopathy were absent. His respiratory system examination revealed decreased breath sounds, decreased vocal resonance and dullness on percussion in the left infrascapular and infra-axillary region. His spleen was palpated 7 cm and the liver 5 cm below the costal margin.
Figure 1: Smooth and shiny red tongue

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Figure 2: Multiple erythematous excoriated papules with scratch marks present over the trunk

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His hemogram revealed a hemoglobin (Hb) of 18.8 g/dL, a hematocrit of 64.4%, erythrocyte count of 8,050,000/mm 3 , total leukocyte count of 13,200/mm 3 , and platelet count of 21,000/mm 3 . His renal, liver and thyroid profile were all normal. His serum erythropoietin level was low at 1.3 mIU/mL (normal 4.3-29 mIU/mL). The result of Janus Kinase 2 (JAK2V617F) mutation test was positive. Bone marrow biopsy showed hypercellularity with prominent erythroid, granulocytic, and megakaryocytic proliferation. His coagulation profile was normal. His serological tests were negative for antinuclear antibodies, antiphospholipid antibodies, hepatitis B virus, hepatitis C virus and human immunodeficiency virus (HIV).

His chest radiograph revealed blunting of left costophrenic angle suggestive of left-sided pleural effusion (PE) [Figure 3]. A left thoracocentesis revealed hemorrhagic pleural fluid that did not clot and did not clear on sequential samples. The pleural fluid was exudative by Light's criteria with cytology showing 2100 cells/mm 3 with predominantly mononuclear cells and a few mesothelial cells and lymphocytes and plenty of RBCs. His pleural fluid adenosine deaminase was 16 U/L (normal < 40 U/L). Pleural fluid cultures were negative for bacteria, fungi, and mycobacterium tuberculosis. Pleural fluid block cytology was negative for malignant cells on two consecutive occasions. A computerized tomographic scan of his chest revealed left-sided pleural effusion [Figure 4]. Further, ultrasonography of abdomen revealed hepatosplenomegaly with no free fluid or no evidence of splenic vein or portal vein thrombosis or splenic infarction and no evidence of a malignant condition.
Figure 3: Chest radiograph revealed blunting of left costophrenic angle

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Figure 4: Computed tomography revealed left-sided pleural effusion

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The diagnosis of PV with thrombocytopenia and left-sided pleural effusion was made. Periodic phlebotomies were done until the hematocrit was decreased to 45%. Myelosuppressive therapy (hydroxyurea 500 mg once a day) was added. Low-dose aspirin could not be added in view of persistent thrombocytopenia that fluctuated between 16,000 and 44,000/mm 3 during the 3 months follow-up period.

  Discussion Top

Revised WHO 2008 criteria for the diagnosis of PV includes two major criteria and three minor criteria. [5] The two major criteria include an increased Hb level (>18.5 g/dL in men or >16.5 g/dL in woman) or other evidence of increased red cell volume and presence of JAK2V617F mutation. The three minor criteria include BM trilineage myeloproliferation, subnormal serum EPO level, endogenous erythroid colony formation (EEC) in vitro. The diagnosis of PV requires the presence of both major criteria and one minor criterion, or the presence of the first major criteria together with two minor criteria. In our case, two major criteria that is, Hb level >18.5 g/dL, presence of JAK2 mutation and two minor criteria that is, BM trilineage hypercellularity and low serum EPO level are fulfilled.

The cause of thrombocytopenia in our patient could be either increased sequestration of platelets as a result of hypersplenism, platelet consumption within the hemorrhagic pleural effusion or increased clearance of platelets as a result of immune thrombocytopenic purpura (ITP). Hypersplenism results in sequestration (pooling) of platelets in an enlarged spleen because of slow passage of platelets through splenic vasculature resulting in thrombocytopenia. [6] Three cases of ITP coexisting with PV were reported by Niscola et al., which responded well to oral steroids and indirectly supported the immunological origin of the low platelet count in these patients. [7] In our patient, platelet count did not improve after oral steroid trial, which did not support the diagnosis of ITP. Platelet associated IgG and/or IgM antibodies could not be done in our patient due to financial constraints.

Pleural fluid examination in our patient was unremarkable for any evidence of malignancy or infection. The etiology of hemorrhagic pleural effusion was unclear even after extensive evaluation for malignancy, infection, autoimmune disease, or pulmonary or splenic thrombosis/infarction. The etiologies of PE in patients with chronic myeloproliferative diseases reported in the literature are infiltration of the pleura at the stage of leukemic transformation, extramedullary hematopoiesis, lung infections or reactive with the presence of mononuclear cells, mesothelial cells, and T lymphocytes. [8] In our patient, the possibility of pleural reaction secondary to intrapleural bleeding was considered after excluding other causes, which might occur as a result of platelet dysfunction. Left-sided pleural effusion secondary to splenic vein thrombosis has been reported in the literature by Warren and Gibbons. [9] But we did not find such evidence in abdominal imaging.

Erythrocytosis, leukocytosis or thrombocytosis or in combination are usually present at the onset of PV. Thrombocytopenia at the onset of PV with hypercellular bone marrow is extremely rare. Cytopenias are noticed in the spent phase (postpolycythemic myeloid metaplasia), an advanced stage of PV characterized by normalization of the red cell mass, increased splenomegaly because of extramedullary hematopoiesis, and collagen fibrosis of the bone marrow (myelofibrosis). [1],[10]

  Conclusion Top

Clinicians or hematologists involved in the care of PV patients should be aware of the unusual occurrence of thrombocytopenia and hemorrhagic pleural effusion as initial presentation of PV.

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Conflicts of Interest

There are no conflicts of interest.

  References Top

Bilgrami S, Greenberg BR. Polycythemia rubra vera. Semin Oncol 1995;22:307-26.  Back to cited text no. 1
Sekhar M, McVinnie K, Burroughs AK. Splanchnic vein thrombosis in myeloproliferative neoplasms. Br J Haematol 2013;162:730-47.  Back to cited text no. 2
Landolfi R, Di Gennaro L, Falanga A. Thrombosis in myeloproliferative disorders: Pathogenetic facts and speculation. Leukemia 2008;22:2020-8.  Back to cited text no. 3
Landolfi R, Cipriani MC, Novarese L. Thrombosis and bleeding in polycythemia vera and essential thrombocythemia: Pathogenetic mechanisms and prevention. Best Pract Res Clin Haematol 2006;19:617-33.  Back to cited text no. 4
Vardiman JW, Thiele J, Arber DA, Brunning RD, Borowitz MJ, Porwit A, et al. The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: Rationale and important changes. Blood 2009;114:937-51.  Back to cited text no. 5
Schatken RV. Hypersplenism: A review and case report. Ann Surg 1952;135:536-42.  Back to cited text no. 6
Niscola P, Giovannini M, Scaramucci L, Palombi M, Tendas A, Cupelli L, et al. Idiopathic thrombocytopenic purpura coexisting with polycythemia vera. Asian J Transfus Sci 2011;5:187-8.  Back to cited text no. 7
[PUBMED]  Medknow Journal  
Alexandrakis MG, Passam FH, Kyriakou DS, Bouros D. Pleural effusions in hematologic malignancies. Chest 2004;125:1546-55.  Back to cited text no. 8
Warren MS, Gibbons RB. Left-sided pleural effusion secondary to splenic vein thrombosis. A previously unrecognized relationship. Chest 1991;100:574-5.  Back to cited text no. 9
Dong XF, Yue LZ, Fu R, Shao ZH. Polycythemia vera transforming to myelofibrosis and then biphenotype acute leukemia. Clin Lab 2014;60:495-9.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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