Blood Diseases: Myeloproliferative

[liveonearth]

MYELOPROLIFERATIVE DISORDERS:
--caused by clonal expansion of pluripotent stem cell in bone marrow (BM) causing abnormal production of erythroid, myeloid, andmegakaryocytic precursors
--reflected in PB smear and CBC results
--multiple BM cell lines usu affected, but one cell line may dominate
--TYPES:
1. Polycythemia Vera
2. Myelofibrosis
3. Essential Thrombocythemia
4. Chronic Myelogenous Leukemia (CML)

PCV = POLYCYTHEMIA VERA
--types:
----1. relative (dt decreased plasma volume, erythopoietin level normal)
----2. secondary (dt hypoxia from smoking, altitude, lung dz, EPO level increased)
----3. primary (malignant, ideopathic, EPO level low to none)
--increased HGB concentration and RBC mass
--increased blood volume and viscousity
--INCIDENCE: 1/1,000,000, 1.4 males: 1.0 females
--mean age at Dx: 60
--S/Sx: weakness, headache, light-headed, fatigue, dyspnea, bleeds easily (epitaxis)
--S/Sx: red face, engorged retinal veins, hepatomegaly, splenomegaly (75%), Raynaud's syndrome
--decreased O2 saturation in periphery
--Dx from LABS: suspect PCV if HCT > 52 in white male or > 47 in black or female, if HGB > 18 in white male or > 16 in female or black, or when simultaneous increase of RBC, WBC & PLT (panmyeolsis)
--LABS: common to see (but not diagnostic): increased uric acid, EPO (erythropoeitin low or undetectable, RBCs > 6 million
--RBC survival time decreases by 25%-->anemia and myelofibrosis may develop
--Bone Marrow: hypercellular
--immature WBC's and RBC's w/ anisocytosis, poikilocytosis: microcytes, elliptocytes and DACROCYTES in peripheral blood (PB)
--thrombocytosis w/ abn morph & if fx abn increased bleeding
--NEUTS with abnormal morphology
--panmyelosis = increased RBCs, WBCs, PLTs
MORE REPEATS??
--immature WBC’s, RBC’s with marked anisocytosis and poikilocytosis
--neutrophils with abnormal morphology may be seen (which abn?)
--thrombocytosis
--expanded blood volume & hyperviscosity-->tissue hypoxia, vessel thrombosis
--Splenomegaly in > 75% of patients
--RBC count > 6,000,000 /mm3
--O2 content of blood increased, but o2 sat decreased
--chronic-->increased cardiac output & increased capillary beds-->decrease tissue hypoxia

What shape is a dacrocyte?

TEARDROP SHAPE

MYELOFIBROSIS
--ideopathic condition in which bone marrow becomes fibrotic
--splenomegaly
--normo/normochromic anemia with DACROCYTES
--myeloid metaplasia: extramedullary hematopoiesis: cells formed in liver and spleen as they were during gestation
--peak incidence 50-70 years
--median survival 10 years from onset
--DX: BM biopsy shows fibrosis, dry aspiration common
--WBC and platelet counts high intially, lower as dz progresses
--LABS show: immature WBCs and RBCs, mild poikilocytosis, polychromatophilia and NRBCs, dacrocytes
--WBCs: initial leukocytosis wiht immature neuts
--may manifest secondary to PV (15-30% of cases), leukemia, lymphoma, multiple myeloma, TB, osteomyelitis, myelodysplastic syndrome MDS, BENZENE exposure, X-rays, gamma rays

THROMBOCYTHEMIA (Essential or primary)
--ideopathic platelet count above 500,000 in the absence of other myeloproliferative dz
--age 50-70
--markedly increased PLT
--BM megakaryocyte hyperplasia
--hemorrhagic or thrombotic tendency (new platelets don't work right)
--LABS: PLT 500,000-1,000,000, normal RBC mass, NO DACROCYTES, NO PHILADELPHIA XSOME (CML)
--CAUSES of SECONDARY (for DDX): acute infx, chronic inflam dz (RA, TB), IDA (iron deficiency anemia), hemolysis, cancer, lymphoma, splenectomy

CML = CHRONIC MYELOGENOUS LEUKEMIA: see LEUKEMIA post, just prior to this one

Comments

[hausfrauatu.livejournal.com]

I have polycythemia in my family. My great-uncle needed to be bled for it. My cousin, who is in her early 20's has Raynaud's syndrome. I'm not sure if they ever connected to dots between the polycythymia and the Raynaud's.

My HGB (Hemoglobin? right?) has been 19. It's usually around 15-17. I get pins and needles quite a bit, which is interesting. I've been told I don't need to worry until menopause.

The last thing I need on my medical records is another chronic condition. I think the bi-polar is enough to make me nearly uninsurable. :P

So how is it treated?

[liveonearth.livejournal.com]

Leeches.

[hausfrauatu.livejournal.com]

Oh dear! o_0

I have donated blood before and I felt awful afterwards. I did it because I had excessive bleeding post partum, and I wanted to give the two units back to the bank. I bled very heavily every time I gave birth, but my G.P. caught it the last time. My HGB was down to 7, which I guess is normal post partum if you are usually at 12 or 14, but for me it was a big difference. I was really weak. My midwife told me I was done for home birthing, for sure, and probably for ANY birthing. Which was fine. Three is more than enough!

I have been feeling more mortal lately. This may give a big hint as to what will do me in eventually. My grandfather died of pancreatic cancer, his brother of leukemia.

Ha! I'm bugging you like you're a DOCTOR:)))) Congrats!

[liveonearth.livejournal.com]

I really appreciate your comments about your body and experience.....helps me to understand and retain if I can attach a condition to a person. You're helping me to become a doc, and for that I thank you.

Some people have Raynaud's without blood conditions.

Nobody feels good when their hemoglobin is 7... glad your GP was on it at last!!

As for what will do you in, who knows if you will live long enough for PCV to kill you! I like that saying about LIFE being a sexually transmitted dz with a 100% mortality rate. If you want to try to live longer, you might investigate what you might do to keep your circulatory system, plus liver and spleen extra strong. There isn't a magic tx for PCV (yet); therapeutic phlebotomy is the state of the art treatment.