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Myeloproliferative vs Myelodysplastic (Myelodysplastic syndrome: …
Myeloproliferative vs
Myelodysplastic
Myeloproliferative Disorders:
Neoplastic proliferations of MATURE myeloid cells
= HIGH WBC
JAK2 mutation
Polycythemia Rubra Vera (PCV) =
Increased RBC
Erythroid production although EPO is LOW
Symptoms
Hyperviscosity + sludging of blood
Thrombosis (majority of morbidity)
Plethoric + cyanotic complexion
Splenomegaly
HTN
Headache, dizziness
Itching and peptic ulceration (histamine release - basophilia)
Hyperuricemia + gout
Complications:
Can evolve to myeloFIBROSIS or AML
Treatment:
Phlebotomy
Labs:
HIGH hematocrit
LOW EPO
+
MPL/Calreticulin
Essential Thrombocytosis =
Increased platelets >600,000
Symptoms
Thrombosis/hemorrage due to dysfunctional platelets
Erhythomelalgia - burning pain + erythema in feet or hands
Labs:
LARGE platelets
Increased mean platelet volume
Increased WBC
DYSMORPHIC MEGAKARYOCYTES in bone marrow
Distinguish from reactive polycythemia caused by inflammation, lung disease, high altitude, tumor causing high EPO
Primary myelofibrosis:
Neoplastic megakaryocytes -->
PDGF + TGFB -->
FIBROSIS --> cytopenia +
extremedullary hematopoiesis
Symptoms
MASSIVE splenomegaly
Anemia
Hypermetabolism (weight loss,
night sweats, hyperuricemia)
Complications:
Infection
Bleeding/thrombosis
Transform into AML
Labs:
Anemia
Leukoerythroblastosis (left shift WBC + nucleated RBC --> tear drop erythrocytes --> giant platelet)
Fibrosis on BM biopsy
BCR-ABL fusion gene t(9;22)
Chronic myelogenous leukemia
c-KIT activating mutation
in MAST CELL
Mastocytosis =
Accumulation of mast cells
Cutaneous mastocytosis (Urticaria Pigmentosa)
SKIN
CHILDREN 👦🏻🧒🏼
Good prognosis
Systemic mastocytosis
Infiltrate skin, lymph nodes, organs, skeleton
ADULTS 👨🏻🧑🏽
Poor prognosis
Symptoms
Abdominal pain
Diarrhea
Itching
Hives
Nasal bleeding
Bone pain
BRAF mutation in
Langerhans cells (dendritic)
Langerhans Cell Histiocytosis
Labs:
Vesicular nuclei with linear grooves
BIRBECK GRANULES
🏸
S-100, CD207 (Lengerin)
Mutated BRAF
Eosinophils
Multisystem LCH
Multisystem infiltrates = most severe
YOUNG children
Eosinophilic granuloma
Unisystem = more common
OLDER children >5 + young adults (up to 30)
Pulmonary
ADULT smokers 🚬
Myelodysplastic syndrome:
Ineffective hematopoiesis =
LOW WBC
Neoplastic HSC can differentiate but are disordered --> peripheral blood cytopenias
Mutations + chromosomal aberrations cause
Incidence INCREASES AGE >50 🧓🏼👵🏻
Idiopathic
Therapy related:
Complication of chemo or radiation
Good prognosis:
5q- (Refractory anemia)
Older women
Megaloblastoid with ringed sideroblasts
Poor prognosis:
7q-, trisomy 8 (myc),
TP53 loss of function
Labs:
Pancytopenia (abnormal megakaryocytes + granulocytes
High MCV
Poikilocytosis (varied RBC shape)
Hypogranular
Pseudo-Pelger-Huet cells
Hypercellular marrow
Megaloblastoid
Ringed sideroblasts
Symptoms
Refractory anemia
Pancytopenia
Infections
Bleeding
Complications:
Transform into AML