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Common Hematologic Maligancies

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jay winegarden

on 27 June 2018

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Transcript of Common Hematologic Maligancies

Case #1
67 yo female presents to your office with complaint of "feeling tired all the time". Initial history does not provide a possible answer and blood work obtained. CBCD indicates normal wbc and plts, but normochromic normocyctic anemia with hgb of 9.0 g/dL and cmp notable for cr of 1.7 , protein of 10 and alb of 2, calcium 8
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Goals:
1. recognition
2. initial diagnostic steps
3. treatment
MGUS vs. Multiple Myeloma
1. MGUS typically with very small monoclonal protein
2.no criteria met for "symptomatic" myeloma: C=elevated calcium
R= renal failure
A= anemia
B= lytic bone lesions
Multiple Myeloma
1. disease of the plasma cell line
2. production of monoclonal proteins that are detected in blood and/or urine
3. cause renal failure, anemia, lytic bone lesions, and hypercalcemia
Common Hematologic Malignancies
Family Medicine Resident Lecture
June 27th 2018

Case #2
50 yo male presents to your office with "feeling tired all the time" x 3 months.History is notable for some mild weight loss, but the patient has been eating more healthy the last several months. History is unremarkable otherwise except for one sick contact at home.

NOW WHAT?
Differential Diagnosis:
1.infection
2. inflammation
3.lymphoma??
Next Steps:
Initial Diagnostic Steps if suspecting lymphoma:
1. CT scan (what about a PET scan?)
2.LDH, ESR, Uric Acid
3. Biopsy -excisional vs needle/core biopsy
4.bone marrow biopsy
Differential Diagnosis:

Multiple Myeloma
MGUS
Amyloidosis
Lymphoma


Monoclonal Gammopathy of Undetermined Significance/MGUS:

Treatment Options:
Hematopoetic Stem Cell Lineage:

Amyloidosis:
Additional Labs/Diagnostics:

1.SPEP
2.serum FLC
3.bone survey
4.24 hour protein for Bence-Jones Protein
Case 1, part 2:
SPEP reveals protein of 3.0 g/dL and serum immunofixation reveals an IgG Kappa. FLC reveals Kappa/lambda light chain raio of 105 and bone survey shows possible lytic lesions in long bones. 24 hour urine indicates free kappa light chain
1. monoclonal proteins seen in many >65 years old
2. have no criteria for meeting myeloma diagnosis
3. treatment is observation...for now
4. risk of transformation to MM is 1%/year
1. divide patients into those who can receive a transplant and those who cannot
2. multiple new chemotherapy drugs developed that can greatly improve survival (a drastic change from only 15 years ago), many of these treatments are very well tolerated (pills)-inluse protesome inhibitors and monoclonal antibodies
3.development and use of bisphosphonates has greatly improved outcome with a decrease in fractures
4.recognition of those whom treatment is needed has improved and lowered morbidity such as renal failure
1. poorly understood disease also of plasma cells leading to deposition of amyloid in tissues: fat, GI tract, kidney heart
2. requires a degree of suspicion of this problem vs MM vs MGUS
3. once heart involvement develops risk of death greatly increased
4. typically that used for MM and role of transplant is not easily defined
Labs indicate normal CBCD and CMP.
PE notable for left posterior cervical lymph node-hard and non mobile.

NOW WHAT?
Case #2, part 2:
Labs indicate elevated LDH and ESR, normal Uric Acid. You order a biopsy (with help of a surgeon). Biopsy reveals "low grade B cell lymphoma". You refer to hem/onc.
Non-Hodgkins Lymphoma
:
Lymphomas are derived from B-cell, T-cell or NK-cell lineages at varying stages o f maturation and with specific genetic signatures

WHO currently recognizes over 60 types of NHL

40% of newly diagnosed cancers in the US are NHL





Most Common Subtypes of Non-Hodgkin Lymphoma

Aggressive
•Diffuse large B-cell lymphoma (DLBCL)
•Anaplastic large cell lymphoma
•Burkitt lymphoma
•Lymphoblastic lymphoma
•Mantle cell lymphoma
•Peripheral T-cell lymphoma
•Transformed follicular and transformed mucosa-associated lymphoid tissue (MALT) lymphomas

Indolent
•Follicular lymphoma
•Cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome)
•Lymphoplasmacytic lymphoma/Waldenström macroglobulinemia
•Marginal zone B-cell lymphoma
•MALT lymphoma
•Small cell lymphocytic lymphoma/chronic lymphocytic leukemia (SLL/CLL)

Treatment Options:
1. chemotherapy
(chemo-immuno therapy)
2.radiation
3.rarely surgery
4.stem cell transplant
5. new horizon is CAR-T

Case 3:
25 yo female presents to you for regular health care maintenance. She notes increased bruising of late. Denies prior history of bleeding and has no family history of blood clotting issues that she is aware of at this time.
Now What
Labs.....
pt/ptt are normal
CBCd indicates normal white blood cell count and hemoglobin, plts are 1,000,000
Essential Thrombocytosis/Myeloproliferative Disorders
1. think about stem cell diagram
2. family of disorders including polycythemia vera, chronic myelogenous leukemia, pirmay myelofibrosis
3. diagnosis based on bone marrow pathology and gene studies
-JAK 2 seen in 100% of PV, approx 50-60% of PM and ET
-t(9;22) diagnostic of CML
Case #3 part 2:
suspecting ET you order JAK 2 study and it is abnormal with mutation of V167F.
-do you need a bone marrow biopsy?
-now what?
-treatment....
-why is this patient bruising?
Case #4:
75 yo male with h/o of hypertension, DM, h/o of alcohol use presents for follow up. CBCd and CMP performed. CMP notable for cr cl 30 cc/min, mildly abnormal liver function and cbc shows increasing anemia with a hgb of 9 g/dL , normal wbc and diff, plts of 80K
Differential Diagnosis:
1,
2.
3.
4.
you order a peripheral blood smear
MDS:
-variety of diseases with variable course all with <20% blasts in bone marrow with variable rates of progression toward acute leukemia
-depending on genetics and degree of cytopenia one may choose treatment
-certain genetic findings dictate treatments- 5q-
-only cure is allo-stem cell transplant
Case #4:
peripheral blood smear:
Bone Marrow:
Acute Luekemia:
1.defined as >20% blasts in peripheral blood or bone marrow
2.WHO classification of M1-M7, but we use that in conjunction with genetic and molecular makers to define prognosis and treatment
3. treatment chemotherapy, consolidation and stem cell transplant
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