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Describe the clinical presentation of MDS.

1) Many patients are asymptomatic at diagnosis and come to their physician for an abnormality on a CDC i.e.
- Anemia
- Neutropenia
- Thrombocytopenia
2) Other patients present with symptoms relating to the above, of which, anemia is the most common


What are the specific physical exam findings that may present with MDS?

- Pallor (anemia)
- Petechiae/ purpura (thrombocytopenia)
- Sweet's Syndrome i.e. Acute Febrile Neutrophilic Dermatosis
- Hepatomegaly/Splenomegaly (relatively uncommon)


What are some of the differential diagnoses to MDS?

1) Vitamin B12 and Folate Deficiency
2) Viral infection e.g. HIV
3) Chemotherapy
4) Alcohol
5) Pb toxicity
6) Benzene
7) Erythropoietic agents e.g. EPO or GM-CSF


What is 5q Syndrome?

MDS associated with a deletion of the long arm of chromosome 5


What are the clinical features of 5q Syndrome>

This is a syndrome seen almost exclusively in older women; it is characterized by:
1) Severe macrocytic anemia
2) Leukopenia
3) Normal to elevated platelet counts
4) Lack of neutropenia


What is CMML?

Chornic Myelomonocytic Leukemia; the overproduction of maturing monocytic cells and sometimes dysplastic neutrophils with:
1) Anemia
2) Thrombocytopenia


What does low intensity treatment entail for MDS?

1) Supportive care
2) Transfusion
3) Growth Factor Support


What is CML?

Neoplastic hyperproliferation of mature myeloid cells, especially granulocytes and their precursors


Describe the presentation of CML.

Symptomatic presentations of CML present most frequently with:
1) Fatigue
2) Bleeding episodes from platelet dysfunction
3) Weight loss
4) Night sweats
5) Abdominal pain/ fullness (LUQ radiating to left shoulder)


What CBC findings are characteristic of CML?

Dramatically increased WBC count with BASOPHILIA being the most specific elevation associated with CML


What are the characteristic features of the chronic phase of CML?

1) Basophilic on WBC diff.
2) Myeloblasts less than 10%
3) Presence of BCR-ABL fusion protein


How is CML diagnosed?

1) CBC/ PBS showing leukocytosis w/ basophilia
2) Bone marrow biopsy showing granulocytic hyperplasia
3) Confirmation with FISH or RT-PCR for BCR/ABL


List the MAJOR criteria for PV.

1) Hb greater than 18.5 and 16.5 for men and women
2) JAK2 mutation


List the MINOR criteria for PV.

1) Bone marrow hypercellularity
2) Low serum EPO


What are the clinical manifestations of ET?

Either increased risk of thrombosis or bleeding events depending on if the overproduced platelets are functional vs. non-functional


What are the diagnostic criteria for ET?

1) Platelet count greater than 450,000
2) BM biopsy showing hyperproliferation of megakaryocyte lineage
3) JAK2 mutation present or rule out reactive thrombocytosis


How is ET treated?

1) Hydroxyurea
2) Anagrelide


What cell line undergoes hyperprolifeation in Primary Myelofibrosis? What factor causes fibrosis?

- Mature myeloid cells i.e. megakaryocytes
- PDGF causes fibrosis of the bone marrow


What are the clinical features of Primary Myelofibrosis?

1) Splenomegaly due to extramedullary myelofibrosis
2) Leukoerythroblastic PBS i.e. tear drop RBCs, nucleated RBCs, immature granulocytes
3) Increased risk of infection, bleeding, and thrombosis


What are the supportive measures used as treatment for Primary Myelofibrosis?

1) Recombinant EPO
2) Iron chelation
3) Splenectomy
4) JAK2 inhibitors
5) Allogenic Stem Cell Transplant*

Note that this is the only treatment currently available that has curative potential.


Generally, how is Multiple Myeloma treated?

1) Chemotherapy
2) Steroids
3) Immunomodulators
4) Bone marrow transplant
5) Bisphosphates/ radiation


How are Plasmacytomas diagnosed?

1) Biopsy proven lesion of the bone or soft tissue WITH:
- Normal bone survey
- No evidence of end organ damage
- Bone marrow with no evidence of plasma cells

Thus, a) demonstrate presence of tumor, b) rule out Multiple Myeloma


What are indications of poor prognosis with Amyloidosis?

1) Cardiac involvement
2) ANS neuropathy
3) Liver involvement with hyperbilirubinemia
4) Lack of suppression of underlying clonal disease


What is the key symptom of Waldenstrom's Macroglobulinemia?

Hyperviscosity Syndrome


What is the treatment for Waldenstrom's Macroglobulinemia?

Chemotherapy and plasmapharesis if symptomatic


What are the major categories of disease that are associated with a higher prevalence of childhood cancer?

1) Immunodeficiency
2) Metabolic disorders
3) Chromosome instability


When a child presents with unexplained petechiae, what are the two disorders that should be high on your differential diagnosis?

1) Leukemia
2) Neuroblastoma


When a child presents with lymphadenopathy that is unresponsive to antibiotics, what are the two disorders that should be high on your differential diagnosis?

1) Leukemia
2) Lymphoma


When a child presents with a pharyngeal mass, what are the three disorders that should be high on your differential diagnosis?

1) Rhabdomyosarcoma
2) Lymphoma
3) Nasopharyngeal carcinoma


When a child presents with a periodontal mass or loose teeth, what are the four disorders that should be high on your differential diagnosis?

1) Langhans Cell Histiocytosis
2) Burkitt's Lymphoma
3) Neruoblastoma
4) Osteosarcoma