Myeloproliferative Neoplasms (MPNs / मायलोप्रोलिफेरेटिव न्यूक्लियोफॉर्म्स) एक समूह की blood cancers (रक्त कैंसर) हैं।
- इसमें bone marrow में abnormal proliferation होती है, जिससे blood cells (RBC, WBC, platelets) की संख्या बढ़ जाती है।
- Chronic condition होती है, धीरे-धीरे progress करती है।
- WHO classification के अनुसार मुख्य MPNs हैं:
- Polycythemia Vera (PV / पॉलिसाइटेमिया वेरा)
- Essential Thrombocythemia (ET / एसेन्शियल थ्रोम्बोसाइटेमिया)
- Primary Myelofibrosis (PMF / प्राइमरी मायलोफिब्रोसिस)
- Chronic Myeloid Leukemia (CML / क्रॉनिक माइलॉइड ल्यूकेमिया)
Myeloproliferative Neoplasms क्या है (What is MPNs)
- Bone marrow में hematopoietic stem cells का uncontrolled growth
- Leads to overproduction of one or more blood cell lines
- Symptoms mostly due to high blood viscosity, splenomegaly, or bleeding/clotting issues
- MPNs can transform into acute leukemia in rare cases
Myeloproliferative Neoplasms कारण (Causes of Myeloproliferative Neoplasms)
1. Genetic Mutations
- JAK2 V617F mutation – most common in PV, ET, PMF
- CALR mutation – common in ET, PMF
- MPL mutation – associated with ET and PMF
2. Stem Cell Abnormalities
- Hematopoietic stem cells grow independently of normal regulation
- Leads to excessive proliferation
3. Risk Factors
- Age >60 years
- Rare familial predisposition
- Environmental factors less clear
Myeloproliferative Neoplasms लक्षण (Symptoms of Myeloproliferative Neoplasms)
Symptoms depend on type of MPN:
1. Polycythemia Vera (PV)
- Headache, dizziness, fatigue
- Reddened face (plethora)
- High blood pressure
- Risk of thrombosis (blood clots)
2. Essential Thrombocythemia (ET)
- Elevated platelets
- Easy bruising, nosebleeds, gum bleeding
- Headache, dizziness
3. Primary Myelofibrosis (PMF)
- Fatigue, weakness
- Enlarged spleen (splenomegaly) – abdominal discomfort
- Night sweats, weight loss
4. Chronic Myeloid Leukemia (CML)
- Fatigue, fever, night sweats
- Enlarged spleen or liver
- Abdominal fullness or pain
Many symptoms overlap; often diagnosed through blood tests and bone marrow biopsy।
Myeloproliferative Neoplasms कैसे पहचाने (Diagnosis of Myeloproliferative Neoplasms)
- Complete Blood Count (CBC) – elevated RBC, WBC, or platelets
- Peripheral Blood Smear – abnormal cell morphology
- Bone Marrow Biopsy – hypercellularity, fibrosis, megakaryocyte proliferation
- Genetic Testing – JAK2, CALR, MPL mutations
- Imaging – ultrasound or CT for splenomegaly
- Other Tests – liver function, uric acid, LDH
Early detection reduces risk of thrombosis, bleeding, and progression to leukemia।
Myeloproliferative Neoplasms इलाज (Treatment of Myeloproliferative Neoplasms)
1. Medications
- Hydroxyurea – control high blood counts
- Interferon-alpha – especially in younger patients
- JAK inhibitors (Ruxolitinib) – for PMF and symptomatic PV
2. Phlebotomy
- Primarily in Polycythemia Vera
- Reduces RBC mass and blood viscosity
3. Low-dose Aspirin
- Reduce risk of blood clots in PV and ET
4. Bone Marrow / Stem Cell Transplant
- Considered in severe PMF or transformation to acute leukemia
5. Supportive Therapy
- Manage anemia, fatigue, infections
- Monitor organ function and platelet count
रोकथाम (Prevention / Risk Reduction)
- Early diagnosis and treatment
- Control cardiovascular risk factors – BP, cholesterol, diabetes
- Avoid smoking
- Regular follow-up with hematologist
सावधानियाँ (Precautions)
- Medication adherence – hydroxyurea, aspirin, or JAK inhibitors
- Monitor for thrombosis, bleeding, and infections
- Report new symptoms promptly – headache, abdominal pain, sudden weakness
- Avoid unnecessary supplements or herbs without doctor approval
FAQs (अक्सर पूछे जाने वाले प्रश्न)
Q1. क्या MPNs cureable हैं?
Mostly chronic conditions; cure possible only via stem cell transplant in selected cases.
Q2. क्या यह genetic है?
Mostly acquired mutations (JAK2, CALR, MPL), rare familial cases exist.
Q3. Life expectancy क्या है?
Depends on type, age, and complications; proper management से near-normal life possible.
Q4. क्या MPNs leukemia में बदल सकते हैं?
हाँ, especially PMF and ET rare cases में acute leukemia में transform हो सकते हैं।
निष्कर्ष (Conclusion)
Myeloproliferative Neoplasms (MPNs / मायलोप्रोलिफेरेटिव न्यूक्लियोफॉर्म्स) chronic blood disorders हैं, जो bone marrow में abnormal proliferation के कारण होती हैं।
Early diagnosis, blood count management, medications, phlebotomy और regular follow-up से complications और progression को significantly reduce किया जा सकता है।
यदि कोई persistent fatigue, unexplained bruising, headache, splenomegaly या abnormal blood test result दिखें, तो तुरंत hematologist से consultation करें