Sandbox:Hannan: Difference between revisions
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|<small>Associationed with polycythemia vera and plasma cell disorders</small> | |<small>Associationed with polycythemia vera and plasma cell disorders</small> | ||
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|Polycythemia vera (PV)<ref name="pmid29194068">{{cite journal |vauthors=Vannucchi AM, Guglielmelli P, Tefferi A |title=Polycythemia vera and essential thrombocythemia: algorithmic approach |journal=Curr. Opin. Hematol. |volume=25 |issue=2 |pages=112–119 |date=March 2018 |pmid=29194068 |doi=10.1097/MOH.0000000000000402 |url=}}</ref><ref name="pmid30252337">{{cite journal |vauthors=Pillai AA, Babiker HM |title= |journal= |volume= |issue= |pages= |date= |pmid=30252337 |doi= |url=}}</ref> | |Polycythemia vera (PV)<ref name="pmid29194068">{{cite journal |vauthors=Vannucchi AM, Guglielmelli P, Tefferi A |title=Polycythemia vera and essential thrombocythemia: algorithmic approach |journal=Curr. Opin. Hematol. |volume=25 |issue=2 |pages=112–119 |date=March 2018 |pmid=29194068 |doi=10.1097/MOH.0000000000000402 |url=}}</ref><ref name="pmid30252337">{{cite journal |vauthors=Pillai AA, Babiker HM |title= |journal= |volume= |issue= |pages= |date= |pmid=30252337 |doi= |url=}}</ref><ref name="pmid30281843">{{cite journal |vauthors=Tefferi A, Barbui T |title=Polycythemia vera and essential thrombocythemia: 2019 update on diagnosis, risk-stratification and management |journal=Am. J. Hematol. |volume=94 |issue=1 |pages=133–143 |date=January 2019 |pmid=30281843 |doi=10.1002/ajh.25303 |url=}}</ref> | ||
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* <small>Constitutional</small> | * <small>Constitutional</small> |
Revision as of 15:03, 16 January 2019
Myeloproliferative neoplasms (MPN) | Clinical manifestations | Diagnosis | Other features | ||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Symptoms | Physical examination | CBC & Peripheral smear | Bone marrow biopsy | Gold standard | Other investigations | ||||||||||
WBCs | RBCs | Platelets | |||||||||||||
Leukocytosis | Blasts | Left shift | Basophilia | Eosinophilia | Monocytosis | Others | |||||||||
Chronic myeloid leukemia |
|
|
Absolute leukocytosis (median of 100,000/µL) | Usually <2% but may present with blast crisis | Left shift with classic myelocyte bulge (myelocytes > metamyelocytes on the blood smear)
|
Absolute basophilia is almost always present | Absolute eosinophilia in 90% of patients | Often present | Anemia | Platelet count is usually normal or elevated but thrombocytopenia indicates advanced stage or an alternative diagnosis |
|
Marrow aspirate & unilateral biopsy with cytogenetics and flow cytometry |
|
||
Chronic neutrophilic leukemia (CNL)[3][4][5] |
|
|
Leukocytosis with chronic neutrophilia | Minimal to none | Significantly less than that of CML | Absent | Absent | Absent | ↑ Lactate dehydrogenase (LDH)
↑ Vitamin B12 levels |
Anemia | Thrombocytopenia | Uniforme and intense hypercellularity with minimal to none fibrosis
Neutrophil toxic granulations and Dohle bodies |
FISH
Imaging for hepatosplenomegaly |
Associationed with polycythemia vera and plasma cell disorders | |
Polycythemia vera (PV)[6][7][8] |
|
|
Normal to mild | None | None | May be present | May be present | Usually absent | ↓ Serum ferritin ↓ Folate levels
↑↑B12 levels |
↑↑ Hb and Hct levels (> 16 and 48%) | None to moderate | Hypercellularity for age with tri-lineage growth
Myelofibrosis (in up to 20% of patients) |
JAK2 mutation studies are diagnostic in 95% of patients | Radioisotope studies
Serum EPO levels LFTs RFTs Imaging studies |
May transform into myelofibrosis or leukemia |
Primary myelofibrosis (PMF)
|
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Essential thrombocythemia (ET) | |||||||||||||||
Chronic eosinophilic leukemia, not otherwise specified (NOS) | |||||||||||||||
MPN, unclassifiable | |||||||||||||||
Mastocytosis |
Other myeloid neoplasm and acute leukemia | Clinical manifestations | Diagnosis | Other features | ||||
---|---|---|---|---|---|---|---|
Symptoms | Physical examination | Lab findings | Bone marrow biopsy | Gold standard | Other investigations | ||
Myeloid/lymphoid neoplasms with eosinophilia and rearrangement of
PDGFRA, PDGFRB, or FGFR1, or with PCM1-JAK2
|
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Myelodysplastic/myeloproliferative neoplasms (MDS/MPN)
|
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Myelodysplastic syndromes (MDS)
|
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Acute myeloid leukemia (AML) and related neoplasms
|
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Blastic plasmacytoid dendritic cell neoplasm | |||||||
Acute leukemias of ambiguous lineage
|
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B-lymphoblastic leukemia/lymphoma
|
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T-lymphoblastic leukemia/lymphoma
|
- 50% of patients are asymptomatic
- Clinical features are generally nonspecific such as left upper quadrant pain, early satiety, fatigue & lethargy (most common presenting symptom), weight loss, and night sweats.
- symptoms of anemia
- bleeding
- priapism
- bone pain
- Abdominal mass or fullness
- Infection
- Headache
- Dyspnoea
- Visual disturbances
- Weakness
- Arthralgia
- Cough
- Malaise
- Dizziness
- Nausea/vomiting
- Ankle oedema
- Mental change
References
- ↑ Savage DG, Szydlo RM, Goldman JM (January 1997). "Clinical features at diagnosis in 430 patients with chronic myeloid leukaemia seen at a referral centre over a 16-year period". Br. J. Haematol. 96 (1): 111–6. PMID 9012696.
- ↑ Thompson PA, Kantarjian HM, Cortes JE (October 2015). "Diagnosis and Treatment of Chronic Myeloid Leukemia in 2015". Mayo Clin. Proc. 90 (10): 1440–54. doi:10.1016/j.mayocp.2015.08.010. PMC 5656269. PMID 26434969.
- ↑ Szuber N, Tefferi A (February 2018). "Chronic neutrophilic leukemia: new science and new diagnostic criteria". Blood Cancer J. 8 (2): 19. doi:10.1038/s41408-018-0049-8. PMC 5811432. PMID 29440636.
- ↑ Maxson JE, Tyner JW (February 2017). "Genomics of chronic neutrophilic leukemia". Blood. 129 (6): 715–722. doi:10.1182/blood-2016-10-695981. PMC 5301820. PMID 28028025.
- ↑ Menezes J, Cigudosa JC (2015). "Chronic neutrophilic leukemia: a clinical perspective". Onco Targets Ther. 8: 2383–90. doi:10.2147/OTT.S49688. PMC 4562747. PMID 26366092.
- ↑ Vannucchi AM, Guglielmelli P, Tefferi A (March 2018). "Polycythemia vera and essential thrombocythemia: algorithmic approach". Curr. Opin. Hematol. 25 (2): 112–119. doi:10.1097/MOH.0000000000000402. PMID 29194068.
- ↑ Pillai AA, Babiker HM. PMID 30252337. Missing or empty
|title=
(help) - ↑ Tefferi A, Barbui T (January 2019). "Polycythemia vera and essential thrombocythemia: 2019 update on diagnosis, risk-stratification and management". Am. J. Hematol. 94 (1): 133–143. doi:10.1002/ajh.25303. PMID 30281843.