Severe thrombocytosis and anemia associated with celiac disease in a young female patient: a case report

Abstract

Introduction: Platelet counts exceeding 1.000 x 103/microl are usually considered secondary to another cause, particularly to chronic myeloproliferative disease (CMPD). Reactive thrombocytosis due to iron deficiency rarely exceeds platelet counts of 700 x 103/microl.

Case presentation: Here we report the case of a young woman presenting with clinical signs of severe anemia. Laboratory findings confirmed an iron-deficiency anemia associated with severe thrombocytosis of 1703 x 103/microl. Macroscopic gastrointestinal and genitourinary tract bleeding was excluded. The excessive elevation of platelets, slightly elevated lactate dehydrogenase and slightly elevated leukocytes along with the absence of other inflammation parameters raised the suspicion of an underlying hematological disease. However, bone marrow evaluation could not prove the suspected diagnosis of a CMPD, especially essential thrombocythemia (ET). In the further clinical course the platelet count returned to normal after raising the hemoglobin to a level close to normal range with erythrocyte transfusion, and normalization of serum iron and decline of erythropoietin. Finally, following small bowel biopsy, despite the absence of typical clinical signs, celiac disease was diagnosed. After discharge from hospital the patient was commenced on a gluten-free diet and her hemoglobin almost completely normalized in the further follow-up period.

Conclusion: This case illustrates the rare constellation of an extreme thrombocytosis most likely secondary to iron deficiency due to celiac disease. This represents, to the best of the authors' knowledge, the highest reported platelet count coincident with iron deficiency. A potential mechanism for the association of iron-deficiency anemia and thrombocytosis is discussed. Even in the presence of 'atypically' high platelets one should consider the possibility of reactive thrombocytosis. Extreme thrombocytosis could emerge in the case of iron deficiency secondary to celiac disease.

Figure 1

Representative photographs of peripheral blood smear and bone marrow smear. (A) In the peripheral blood smear there is an obviously increased platelet count with the presence of micro- and macro-platelets (white arrows). Erythrocytes reveal an anisocytosis and pronounced anulocytosis (black arrow). Note the presence of regular erythrocytes which represents the erythrocyte population after successful transfusion of two erythrocyte concentrates. (B) In the bone marrow smear there is a clearly increased count of juvenile megakaryocytes (white arrows). A representative megakaryocyte is shown at higher magnification in the inlet. No clustering of megakaryocytes is evident in this section.

Figure 2

Course of hematological parameters. This figure summarizes the results of relevant laboratory testing. Clearly, concomitant with the transfusion of a total of erythrocyte concentrates (each arrow indicates two concentrates) there was a significant increase in hemoglobin, reticulocytes and serum iron. In parallel, the level of erythropoietin declined rapidly, leucocytes normalized and, finally, even the excessively elevated platelet count returned to the normal range without any specific treatment.