Hematology Anemia, Lymphoma, MM, Plt, etc Flashcards
What is Multiple Myeloma?
A monoclonal neoplasm of the plasma cells characterized by monoclonal Ig overproduction. (M protein).
Classiffied under a group of diseases known collectively as the monoclonal gammopathies or plasma cell dyscrasias.
What are the 3 types of monoclonal gammopathies or plasma cell dyscrasias?
Multiple Myeloma;
- IgG>IgA, >10% in bone marrow
Presentation = CRAB
Complications = 1°amyloidosis, Renal failure, Infections
Waldenström macroglobulinemia
- IgM, >10% in bone marrow
Presentation; Hyperviscosity syndrome
Complications; Thrombotic
MGUS (monoclonal gammopathy of undetermined significance)
- variable Ig, <10% in bone marrow
Presentation; asymptomatic
Complications; transition to MM
What is the median age and common gender for developing Multiple Myeloma?
70 years
Males
*blacks have twice the incidence
Clinical presentation of Multiple Myeloma
CRAB
¡Calcium, Renal, Anemia, Bone pain
Anemia -Normocytic, normochromic
Replacement of normal marrow
Factors released from tumor cells inhibit hematopoiesis, with Renal failure there is also decreases EPO
Bone pain - precipitated by movement
X-ray = typical punched out lesions
Due to proliferation of tumor cells within bone, ¡osteoclast activity (RANK-L expressed by tumor cells) +decreased osteoblast activity
Hypercalcemia- due to increased ca2+ release from bone, later the renal failure contributes.
Renal disease - Toxic effect of filtered light chains = renal failure (cast nephropathy)/ type II RTA
Amyloidosis or light chain deposition = Nephrotic syndrome
Hypercalcemia, Urate nephropathy
Decreased anion gap because M protein is cationic resulting in chloride retention.
Reccurent infections
- relative hypogammaglobulinemia. The monoclonal abnormal plasma cells suppress normal immunoglobulins.
**S.pneumonia, Klebsiella S.aureus (pneumonia)
***E.coli (Pyelonephritis )
Hypercalcemia can cause neurologic problems e.g lethargy, weakness, depression e.t.c
POEMS syndrome
(Polyneuropathy, Organomegaly, Endocrinopathy, M protein, Skin changes)
+PEST (papilledema, extravascular volume overload, sclerotic bone lesions, Thrombocytosis)
Describe primary amyloidosis
Light chain (AL) Amyloidosis
The abnormal monoclonal plasma cells produce abnormal light chains. They come together to form amyloid deposits causing damage to organs. In urine they are called Bence Jones proteins
Diagnosis of Multiple Myeloma
Criteria;
1. Serum(>30g/l) or Urine M protein
2. Bone marrow plasma cells >10%
3. Myeloma related organ failure
*Serum electrophoresis
*Urine electrophoresis
*Bone marrow Biopsy
CRAB
**X-ray= lytic lesions
**Labs = Hypercalcemia >1.5g/l, creatinine>2mg/dl, Hgb<10g/dl
***Immunofixation =lambda/kappa
Management for Multiple Myeloma
Treatment not indicated for MGUS
Age<70, good performance status
= Transplant eligible
**High dose chemo--> autologous-HSCT--> maintenence therapy**
Chemotherapy = VTD regimen
- Bortezomib, Thalidomide, Dexamethasone.
What is Hodgkin Lymphoma?
Malignant disorder of lymphoid cells that reside predominantly in the lymphoid tissues.
Characterized by Reed-Sternberg (RS) cells
What is the age and gender that Hodgkin Lymphoma frequently occurs?
Bimodal distribution
Age 15-35 and Age >50
Male
Clinical presentation of Hodkin Lymphoma
***Orderly anatomic spread! (Contiguous spread)
*Lymphadenopathy*
- cervical/supraclavicular
+/-mediastinal
Constitutional symptoms
- B symptoms
*weightless, night sweats, fever*
- periodic, recurrent
*Pel-Ebstein Fever*
- Pruritus
Others;
- Hypercalcemia
- Immune Hemolytic anemia
- Immune Thrombocytopenia
- preural effusion from extensive mediastinal adenopathy
- Alcohol = pain in lymph nodes
- paraneoplastic cerebellar degeneration
- Nephrotic syndrome
- erythema nodosum
Diagnosis of Hodgkin Lymphoma
Physical examination
- include lymph nodes, liver/spleen,
Excisional lymph node biopsy
- Reed-Sternberg cells <1% in the background of non-neoplastic polyclonal inflammatory cells
Classic = Bilobed nucleus with prominent nucleoli (owl's eye)
If CNS then Lumbar puncture
Labs ;
*Hypercalcemia, anemia, thrombocytopenia,*
RS cells on Flow Cytometry
CD15+,CD30+, CD20-
Chest, abdominal, pelvic CT
PET-CT (best for staging and follow-up)
Describe WHO classification for Hodgkin Lymphoma
Classical vs Non-classical
Classic
*Lymphocyte-rich 5%
- Abundant normal appearing
(good prognosis)
*Nodular rich 60-80%
-Collagen bands on histology
Mediastinal involvement
Young adult, Female predominance
*Mixed cellularity 15-30%
- Pleomorphic cells on histology
Older age, male predominance
(intermediate prognosis)
*Lymphocyte depletion <1%
- Diffuse fibrosis and large numbers of RS cells
seen in HIV, worst prognosis
***Non-Classical***
Nodular Lymphocytic predominant (NLP) 5%
-involves peripheral lymph nodes; "popcorn cells", CD20+
(good prognosis)
Describe the Ann-Arbor staging system for both Hodgkin and non-Hodgkin lymphoma
Stage I
- single lymph node region or lymphoid structure
(spleen, thymus, waldeyer's ring)
Stage II
- involvement of 2 or more lymph node regions on same side of diaphragm.
Stage III
- involvement of lymph node regions on both sides of the diaphragm.
III1- Subdiaphragmatic (spleen, splenic hilar nodes, celiac nodes)
III2- Subdiaphragmatic (paraarotic, iliac, or mesenteric nodes plus III1)
Stage IV
- involvement of extranodal sites
Liver or bone marrow
A no symptoms
B - B symptoms
E - involvement of extralymphatic tissue excluding liver and BM
What is Non-Hodgkin Lymphoma?
Malignant disorders of the lymphoid cells that reside in lymphoid tissues.
Median age and gender for non-hodgkin lymphoma
Median age is 65
Male
Primary and secondary immunodeficiency predisposes to non-hodgkin lymphoma
Patient presentation for NHL
Lymphadenopathy =multiple lymph nodes involved in a diffused manner.
Nodal and extranodal disease with non-contiguous spread
B symptoms less common than HL
***Pruritus
Classification for NHL (lymphoid malignancies)
Mature B cell (85%) vs Mature T cells (15%)
Mature B cell;
1. Diffuse large B cell lymphoma
*most common
2. Follicular lymphoma
*2nd most common
3. CLL/small lymphocytic lymphoma
4. Mantle cell lymphoma t(11;14)
Translocation of cyclin D1 & heavy chain Ig ****CD5+
(Very aggressive)
5. Marginal zone lymphoma t(11;18)
Associated with chronic inflammation.
Nodal, extranodal (MALT) or splenic
6. Burkitt lymphoma t(8;14) ¡c-Myc
Endemic (Africa) - jaw mass EBV
Sporadic (America) - abdominal/pelvic mass EBV(20%)
HIV related
7. Primary CNS lymphoma
EBV related; associated with HIV
**AIDS defining illness (CD4<50)
Single ring enhancing lesion (MRI)
Mature T Cell
1. Adult T-Cell lymphoma
Caused by HTLV-1 (iv drug use)
2. Mycosis Fungoides
Skin patches and plaques (cutaneous T cell lymphoma)
Progression to T cell leukemia (Sézary syndrome)
