giant ribbon-like platelets mimicking microfilaria in a ......tushar sehgal, prashant sharma*, shano...

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IMAGES AND DIAGNOSIS Giant ribbon-like platelets mimicking microfilaria in a JAK2-positive myeloproliferative neoplasm Tushar Sehgal, Prashant Sharma * , Shano Naseem, Neelam Varma Department of Hematology, Level 5, Research Block A, Postgraduate Institute of Medical Education and Research, Sector 12, Chandigarh 160012, India Received 7 August 2015; accepted 28 September 2015 Available online 27 October 2015 A 65-year-old man presented with weight loss, easy fati- gability, night sweats, and massive splenomegaly, ongoing for 2 months. There was no history of blood transfusions and no lymphadenopathy was detected. Hemoglobin was 125 g/L, total leukocyte count was 6.9 10 9 /L, and platelets were 358 10 9 /L. Blood film showed normocytic nor- mochromic red cells and 85% neutrophils with a mild shift- to-the-left (2% metamyelocytes). A few long ribbon-like eosinophilic fragments were present (Figure 1) that were reminiscent of microfilaria (larval forms of various filarial nematodes) at scanner magnification. However, the oil immersion objective revealed cytoplasmic granularity. This, along with the absence of typical cephalic space and multi- ple nuclear columns of the nematode larvae, confirmed the structures to be simply extremely elongated platelets. Sim- ilar, but shorter, elongated platelets were also otherwise abundant (Figure 1, inset). A literature search revealed that platelet-strings or ribbons are normally shed by megakary- ocytes into marrow sinuses and these then fragment into numerous platelets. 1 Our patient’s bone marrow was markedly hypercellular with panmyelosis and especially prominent megakaryocytic hyperplasia and clustering (Figure 2). The megakaryocytes varied from predominantly normal to a few hypolobate and occasional hyperlobate forms (Figure 2). Reticulin was not increased (European Myelofibrosis Network, EUMNET grade, 0–3). Amplification refractory mutation system- polymerase chain reaction revealed heterozygous state for the JAK2 V617F mutation (Figure 3). Reverse transcrip- tase-polymerase chain reaction was negative for the BCR-ABL1 fusion gene. Filariasis is endemic in many parts of India 2 and coexis- tent malignant and parasitic disorders are well-described. 3,4 A high index of suspicion is therefore useful for tropical pathologists; however, the possibility of misdiagnosis in the current case was unlikely once close examination was done by experts well-versed in parasite morphology. This case, apart from the unusual platelet morphology, also illustrates the practical dilemmas faced by hematopathologists in the classification of JAK2-positive myeloproliferative neoplasms with overlapping clinical, morphological, and hematological findings. Our patient does not meet blood count criteria for overt polycythemia vera (PV) or essential thrombocythemia. He may fit into primary myelofibrosis (a prefibrotic cellular stage) or a prepolycythemic stage of PV. The large spleen, however, argues against prefibrotic primary myelofibrosis, while http://dx.doi.org/10.1016/j.hemonc.2015.09.004 1658-3876/Ó 2015 King Faisal Specialist Hospital & Research Centre. Published by Elsevier Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). * Corresponding author. Tel.: +91 8872016123. E-mail address: [email protected] (P. Sharma). Hematol Oncol Stem Cell Ther (2016) 9, 8081 Available at www.sciencedirect.com ScienceDirect journal homepage: www.elsevier.com/locate/hemonc

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Page 1: Giant ribbon-like platelets mimicking microfilaria in a ......Tushar Sehgal, Prashant Sharma*, Shano Naseem, Neelam Varma Department of Hematology, Level 5, Research Block A, Postgraduate

Hematol Oncol Stem Cell Ther (2016) 9, 80–81

Avai lab le at www.sc iencedi rect .com

ScienceDirect

journal homepage: www.elsevier .com/ locate /hemonc

IMAGES AND DIAGNOSIS

Giant ribbon-like platelets mimickingmicrofilaria in a JAK2-positivemyeloproliferative neoplasm

http://dx.doi.org/10.1016/j.hemonc.2015.09.0041658-3876/� 2015 King Faisal Specialist Hospital & Research Centre. Published by Elsevier Ltd.This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

* Corresponding author. Tel.: +91 8872016123.E-mail address: [email protected] (P. Sharma).

Tushar Sehgal, Prashant Sharma *, Shano Naseem, Neelam Varma

Department of Hematology, Level 5, Research Block A, Postgraduate Institute of Medical Education and Research,Sector 12, Chandigarh 160012, India

Received 7 August 2015; accepted 28 September 2015Available online 27 October 2015

A 65-year-old man presented with weight loss, easy fati-gability, night sweats, and massive splenomegaly, ongoingfor 2 months. There was no history of blood transfusionsand no lymphadenopathy was detected. Hemoglobin was125 g/L, total leukocyte count was 6.9 109/L, and plateletswere 358 109/L. Blood film showed normocytic nor-mochromic red cells and 85% neutrophils with a mild shift-to-the-left (2% metamyelocytes). A few long ribbon-likeeosinophilic fragments were present (Figure 1) that werereminiscent of microfilaria (larval forms of various filarialnematodes) at scanner magnification. However, the oilimmersion objective revealed cytoplasmic granularity. This,along with the absence of typical cephalic space and multi-ple nuclear columns of the nematode larvae, confirmed thestructures to be simply extremely elongated platelets. Sim-ilar, but shorter, elongated platelets were also otherwiseabundant (Figure 1, inset). A literature search revealed thatplatelet-strings or ribbons are normally shed by megakary-ocytes into marrow sinuses and these then fragment intonumerous platelets.1

Our patient’s bone marrow was markedly hypercellularwith panmyelosis and especially prominent megakaryocytic

hyperplasia and clustering (Figure 2). The megakaryocytesvaried from predominantly normal to a few hypolobateand occasional hyperlobate forms (Figure 2). Reticulin wasnot increased (European Myelofibrosis Network, EUMNETgrade, 0–3). Amplification refractory mutation system-polymerase chain reaction revealed heterozygous state forthe JAK2 V617F mutation (Figure 3). Reverse transcrip-tase-polymerase chain reaction was negative for theBCR-ABL1 fusion gene.

Filariasis is endemic in many parts of India2 and coexis-tent malignant and parasitic disorders are well-described.3,4

A high index of suspicion is therefore useful for tropicalpathologists; however, the possibility of misdiagnosis inthe current case was unlikely once close examination wasdone by experts well-versed in parasite morphology.

This case, apart from the unusual platelet morphology,also illustrates the practical dilemmas faced byhematopathologists in the classification of JAK2-positivemyeloproliferative neoplasms with overlapping clinical,morphological, and hematological findings. Our patientdoes not meet blood count criteria for overt polycythemiavera (PV) or essential thrombocythemia. He may fit intoprimary myelofibrosis (a prefibrotic cellular stage) or aprepolycythemic stage of PV. The large spleen, however,argues against prefibrotic primary myelofibrosis, while

Page 2: Giant ribbon-like platelets mimicking microfilaria in a ......Tushar Sehgal, Prashant Sharma*, Shano Naseem, Neelam Varma Department of Hematology, Level 5, Research Block A, Postgraduate

Figure 2 The bone marrow biopsy showed near-maximalcellularity with panmyelosis (hematoxylin and eosin, 100).Megakaryocytic proliferation and nuclear atypia wereprominent (inset; hematoxylin and eosin, 400).

Figure 1 A giant ribbon-like platelet in circulation. (May-Grunwald Giemsa, 400). Several other elongated, albeit shorterplatelets were also seen (inset; May-Grunwald Giemsa, 1000). Pa�ent Nega�ve Marker Nega�ve

1 2 4 3

Internal control band

Figure 3 A 2% agarose gel resolving the products ofamplification refractory mutation system-polymerase chainreaction for a JAK2 V617F mutation. The patient data in Lane1 shows two bands indicating heterozygosity for the mutation.The negative cases in Lanes 2 and 3 do not show the mutantband (arrow).

Giant platelets in MPN mimicking microfilaria 81

the heterozygous state for JAK2 mutation is less likely inPV. The case was, therefore, finally diagnosed as myelo-proliferative neoplasms-unclassifiable; with the suggestionto carefully follow up his blood counts and clinical status.

Conflicts of interest

The authors have no conflicts of interest to declare.

References

1 Shafer J. Platelet Ribbon, Normal blood. In: Bloodline Image Atlas(website). Carden Jennings Publishing Co. Ltd. <http://image.bloodline.net/stories/storyReader%24783.html> [Date accessedNovember 10, 15].

2 Sharma P, Kumar N, Jain P, Gur R, Jain S. Chronic wuchereriasispresenting as a vaginoperineal fistula: report of a case withaspiration cytologic diagnosis. Acta Cytol. 2005;49:335–8.

3 Sharma P, Varma N. Pancytopenia following vivax malaria in a CLLpatient. Blood 2013;122:3098.

4 Sharma P, Tyagi S. An unusual cause of eosinophilia in AML-M4without the Inv(16) abnormality. J Blood Disord Transfus2010;1:104.