A Case of Philadelphia Chromosome Positive Myeloproliferative Neoplasm in a Pregnant Woman with Unusual Primary Myelofibrosis Features

نویسندگان

  • Jason Koshy
  • Jack Alperin
  • Bagi Jana
  • Avi Markowitz
  • You-Wen Qian
چکیده

Myeloproliferative neoplasms (MPNs) are traditionally separated into BCR-ABL-positive chronic myeloid leukemia (CML), and BCR-ABL-negative MPNs including primary myelofibrosis (PMF), essential thrombocythemia (ET), and so forth. One of the diagnostic requirements for PMF and ET is the absence of the Philadelphia chromosome, while its presence is almost universally indicative of CML. However, a diagnostic dilemma arises when Philadelphia chromosome-positive MPNs lack the majority of the typical features seen in CML. Some of these classic CML features include basophilIa, marked leukocytosis, neutrophils left-shift with myelocytes bulge, and "dwarf" megakaryocytes. Presented here is a case of a 32-year-old pregnant patient who did not have typical morphologic findings for CML, and yet the Philadelphia chromosome was positive. The patient demonstrated some pathologic features that are commonly presented in PMF that included bone marrow reticulin fibrosis, leukoerythroblastosis, splenomegaly, and increased serum lactate dehydrogenase.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Myeloproliferative syndromes: a unique presentation of chronic myelogenous leukemia (CML) as a primary tumor of bone.

A 45-yr-old female presented with a rapidly enlarging bony tumor that was eventually identified as a Philadelphia chromosome (Ph1)-positive myeloproliferative disorder with extramedullary blastic transformation. This transformation occurred in the absence of demonstrable chronic or acute leukemic phase. She had no history of a chronic or prodromal illness in spite of a bone marrow biopsy showin...

متن کامل

Myeloproliferative Syndromes : A Unique Presentation of Chronic M

A 45-yr-old female presented with a ness in spite of a bone marrow biopsy rapidly enlarging bony tumor that was showing myelofibrosis and liver biopsy eventually identified as a Philadelphia documenting extramedullary hematopoiechromosome (Ph’)-positive myeloprolifersic. This case represents a unique constelative disorder with extramedullary blastic lation of features of the myeloproliferative ...

متن کامل

Detection of Jak2 V617f Mutation, Secondary to the Presence of Bcr-Abl1 Translocation in a Patient with Chronic Myeloid Leukemia: Report of a Case and Review of the Literature

The Myeloproliferative Neoplasms (MPN) are classified as four major diseases: Chronic Myeloid Leukemia (CML), Polycythemia Vera (PV), Primary Myelofibrosis (PMF) and Essential Thrombocythemia (ET) [1]. The translocation t(9;22)(q34;q11) produces the Philadelphia chromosome and causes the BCR-ABL1 transcript; this alteration is commonly found in CML [1]. In 2008 the World Health Organization (WH...

متن کامل

[Utility of bone marrow biopsy in the diagnosis of myeloproliferative neoplasm].

A diagnostic approach of myeloproliferative neoplasms, according to the 2008 WHO classification system for hematological malignancies, has to consider clinical, molecular, and cytogenetic information as well as bone marrow histology. A diagnosis of chronic myeloid leukemia requires the presence of BCR-ABL-1, and the Philadelphia chromosome-negative (Ph-1-negative) myeloproliferative neoplasms c...

متن کامل

Evaluation of JAK2V617F mutation prevalence in myeloproliferative neoplasm by AS-RT-PCR

Abstract Objective JAK2 is a non-receptor tyrosine kinase that plays a major role in myeloid disorders. JAK2V617F mutation is characterized by a G to T transverse at nucleotide 1849 in exon 12 of the JAK2 gene, located on the chromosome 9p, leading to a substitution of valine to phenylalanine at amino acid position 617 in the JAK2 protein. Methods In this study we evaluated RNA from 89 pati...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:

دوره 2013  شماره 

صفحات  -

تاریخ انتشار 2013