CONCOMITANT LATENT POLYCYTHEMIA VERA AND MGUS.

Objective: With the introduction of changes in the diagnostic criteria for polycythemia vera (PV) in the 2016 WHO classification, it became necessary to revise the diagnosis in some patients. Cases with latent (masked) polycythemia vera (LPV) were identified. Bone marrow trepanobiopsy takes one of t...

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Autores principales:	Fidan KHALILOVA, Azer KERIMOV, Zohra ALIMIRZAYEVA
Formato:	article
Lenguaje:	EN
Publicado:	Elsevier 2021
Materias:	Diseases of the blood and blood-forming organs RC633-647.5
Acceso en línea:	https://doaj.org/article/f1138c80b1a948e9b3eb8bcd152f8f53
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spelling	oai:doaj.org-article:f1138c80b1a948e9b3eb8bcd152f8f532021-11-10T04:35:25ZCONCOMITANT LATENT POLYCYTHEMIA VERA AND MGUS.2531-137910.1016/j.htct.2021.10.1025https://doaj.org/article/f1138c80b1a948e9b3eb8bcd152f8f532021-11-01T00:00:00Zhttp://www.sciencedirect.com/science/article/pii/S253113792101172Xhttps://doaj.org/toc/2531-1379Objective: With the introduction of changes in the diagnostic criteria for polycythemia vera (PV) in the 2016 WHO classification, it became necessary to revise the diagnosis in some patients. Cases with latent (masked) polycythemia vera (LPV) were identified. Bone marrow trepanobiopsy takes one of the most important place in the differential diagnosis of LPV with other myeloproliferative diseases.We describe a case with coexistence of LPV and MGUS in a patient at the onset of the disease. Case report: Patient F.I., aged 62, was admitted with complaints of burning sensation in both feet, pain in the left lower extremity, back pain, nocturia 2-3 times per night and weight loss of 6-7 kg.Lumbar and whole spinal MRI revealed changes in the intensity of the medullary signal, mild decrease in the height of L2-L3 and T10 . EMG revealed polyneuropathy.PET showed a moderate uptake of FDG in the localization of the bone marrow. The spleen was enlarged-size-157 mm. Methodology: Laboratory findings: hemogram-WBC-11.15 × 103/µL, Hgb-15g / dL, HCT-48%, PLT-604 × 103/µL. Bone marrow biopsy, imprint, aspiration revealed moderately hipercellular bone marrow with increasing in all 3 series, groupings in megakaryocytes, containing limited (3-4%) kappa monoclonal plasma cell population; moderately increasing reticulin fibers (grade 1 according to WHO). Karyotype 46, XY; multiple myeloma FISH panel: translocation 4; 14 and translocation 11; 14 (+). JAK2V617F-50.48% (+). Results: The key point in the diagnosis was trilineage hyperplasia of the bone marrow,because the reticulin fibrosis may occurs in 20% of PV cases. Thus, the patient was diagnosed with LPV. Due to the detection of plasma cells in the bone marrow (3-4%), kappa light chains, with the diagnosis of LPV, the diagnosis of MGUS was established.The patient was prescribed ASS 100 mg per os, Hydrea at a dose of 500 mg every other day. For MGUS, the “wait and watch” tactic was chosen. Conclusion: In the diagnosis of LPV, along with molecular genetic research, trepanobiopsy of the bone marrow plays a leading role. The possibility of a combination of myeloproliferative and other diseases should not be ruled out.Fidan KHALILOVAAzer KERIMOVZohra ALIMIRZAYEVAElsevierarticleDiseases of the blood and blood-forming organsRC633-647.5ENHematology, Transfusion and Cell Therapy, Vol 43, Iss , Pp S38-S39 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Diseases of the blood and blood-forming organs RC633-647.5
spellingShingle	Diseases of the blood and blood-forming organs RC633-647.5 Fidan KHALILOVA Azer KERIMOV Zohra ALIMIRZAYEVA CONCOMITANT LATENT POLYCYTHEMIA VERA AND MGUS.
description	Objective: With the introduction of changes in the diagnostic criteria for polycythemia vera (PV) in the 2016 WHO classification, it became necessary to revise the diagnosis in some patients. Cases with latent (masked) polycythemia vera (LPV) were identified. Bone marrow trepanobiopsy takes one of the most important place in the differential diagnosis of LPV with other myeloproliferative diseases.We describe a case with coexistence of LPV and MGUS in a patient at the onset of the disease. Case report: Patient F.I., aged 62, was admitted with complaints of burning sensation in both feet, pain in the left lower extremity, back pain, nocturia 2-3 times per night and weight loss of 6-7 kg.Lumbar and whole spinal MRI revealed changes in the intensity of the medullary signal, mild decrease in the height of L2-L3 and T10 . EMG revealed polyneuropathy.PET showed a moderate uptake of FDG in the localization of the bone marrow. The spleen was enlarged-size-157 mm. Methodology: Laboratory findings: hemogram-WBC-11.15 × 103/µL, Hgb-15g / dL, HCT-48%, PLT-604 × 103/µL. Bone marrow biopsy, imprint, aspiration revealed moderately hipercellular bone marrow with increasing in all 3 series, groupings in megakaryocytes, containing limited (3-4%) kappa monoclonal plasma cell population; moderately increasing reticulin fibers (grade 1 according to WHO). Karyotype 46, XY; multiple myeloma FISH panel: translocation 4; 14 and translocation 11; 14 (+). JAK2V617F-50.48% (+). Results: The key point in the diagnosis was trilineage hyperplasia of the bone marrow,because the reticulin fibrosis may occurs in 20% of PV cases. Thus, the patient was diagnosed with LPV. Due to the detection of plasma cells in the bone marrow (3-4%), kappa light chains, with the diagnosis of LPV, the diagnosis of MGUS was established.The patient was prescribed ASS 100 mg per os, Hydrea at a dose of 500 mg every other day. For MGUS, the “wait and watch” tactic was chosen. Conclusion: In the diagnosis of LPV, along with molecular genetic research, trepanobiopsy of the bone marrow plays a leading role. The possibility of a combination of myeloproliferative and other diseases should not be ruled out.
format	article
author	Fidan KHALILOVA Azer KERIMOV Zohra ALIMIRZAYEVA
author_facet	Fidan KHALILOVA Azer KERIMOV Zohra ALIMIRZAYEVA
author_sort	Fidan KHALILOVA
title	CONCOMITANT LATENT POLYCYTHEMIA VERA AND MGUS.
title_short	CONCOMITANT LATENT POLYCYTHEMIA VERA AND MGUS.
title_full	CONCOMITANT LATENT POLYCYTHEMIA VERA AND MGUS.
title_fullStr	CONCOMITANT LATENT POLYCYTHEMIA VERA AND MGUS.
title_full_unstemmed	CONCOMITANT LATENT POLYCYTHEMIA VERA AND MGUS.
title_sort	concomitant latent polycythemia vera and mgus.
publisher	Elsevier
publishDate	2021
url	https://doaj.org/article/f1138c80b1a948e9b3eb8bcd152f8f53
work_keys_str_mv	AT fidankhalilova concomitantlatentpolycythemiaveraandmgus AT azerkerimov concomitantlatentpolycythemiaveraandmgus AT zohraalimirzayeva concomitantlatentpolycythemiaveraandmgus
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CONCOMITANT LATENT POLYCYTHEMIA VERA AND MGUS.

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