Pathology > Basic Hematology > White Cell Disorders > Immunosecretory Disorders: Questions

Immunosecretory Disorders: Questions

HEMATOPATHOLOGY

The arrow points to:

A. Albumin
B. a 1 globulin
C. a 2 globulin
D.b globulin
E. monoclonal protein

 

A 78-year-old black man, a retired chemistry professor, comes to you because of lumbosacral vertebral pain. There is no hepatomegaly. Cardiac exam is normal and there is no evidence of arthritis. His Hct, MCV, WBC and differential are normal. Liver enzymes are normal, but total serum protein is mildly elevated. The serum Ca is normal. You find no protein in his urine. A marrow aspirate&biopsy showed 6% plasma cells (normal=0-5%). A roetgenographic survey was negative. His serum protein electrophoresis is shown at right.

What is the most likely diagnosis based on the history and laboratory findings?

A. Waldenstrom's macroglobulinemia
B. Amyloidosis
C. Myeloma
D. Monoclonal Gammopathy of Unknown Significance
E. Heavy Chain Disease

A 72-year-old black woman, a retired school teacher, who you see for hypertension and mild arthritis, complains of mid-back and hip pain for 4-5 months. Two days ago she fractured her arm trying to lift her 15 month old grandson. Her Hct is 0.30; the WBC 3.8 x109/L with a normal differential. Serum Ca is elevated at > 12 mg/dL.Urine protein is 3+ positive. Her serum protein electrophoresis is shown at right. Physical examination shows no hepatosplenomegaly, nor lymphadenopathy.

What is the most likely primary diagnosis based on the history and laboratory findings?

A. Waldenstrom's macroglobulinemia
B. Amyloidosis
C. Myeloma
D. Monoclonal Gammopathy of Unknown Significance
E. Heavy Chain Disease

What additional studies are needed to confirm your differential diagnosis?

 

Immunosecretory Disorders: Myeloma

 

Immunoelectrophoresis (IEP)is important to confirm and define an apparent monoclonal band seen on SPEP.

Observe the shape of the IgG, IgA, & IgM precipitin arcs between the anti polyvalent Ig and the Control. Compare the arcs of the Pt with the Control looking for the presence of abnormal arcs and matching the abn arcs with those on the type specific heavy & light antisera.

Abn-Igs usually have skewed* or split arcs compared to normal (symmetrical due to Ig molecule heterogeneity). Usually abn-Igs are in excess and/or to the exclusion of normal. Here the Pt has an IgG in excess, but little IgA, or IgM. The k arc is in exess and matches the abn *arc seen against the anti polyvalent Ig. This is a k monoclonal IgG.

 

 

Immunoelectrophoresis (IEP) of a second case in which a monoclonal band was seen on SPEP.

Again compare the shape of the IgG, IgA, & IgM precipitin arcs between the anti polyvalent Ig and the Control.Look for abnormal arcs and match them with those on the type specific heavy & light antisera.

Here the abn-Ig has a split* arc compared to the Pts normal protein# (symmetrical due to Ig molecule heterogeneity). The *arc is identical to the l arc and the #arc with the k arc. The l arc is in exess and matches the abn *arc seen against the anti polyvalent Ig. This is a l monoclonal IgG.

This should be confirmed with immunofixation electrophoresis.

 

 

Immunosecretory Disorders

 

Immunofixation electrophoresis (M-fix) is useful for confirmation and specific identification of monoclonal bands. Antibodies against immunoglobulins, in this case IgG, k, and l, are used to identify the protein bands in the serum protein electrophoresis (SPEP) as an IgG l monoclonal band.

 

HEMATOPATHOLOGY

A 24-year-old woman, recently returned from graduate religious studies in India, comes to you because of soft tissue swelling of her lower extremities. She was treated with phenacetin for "arthritis" while in India. She also noted tenderness of both wrists and some numbness/sensation loss in her hands. On physical exam the soft tissue of her wrists is slightly swollen and firm. Laboratory studies show the Hct is 0.38 and the WBC 8.4x109/L with a normal differential. Her urine is 3+ positive for protein. There is no lymphadenopathy or hepatosplenomegaly. A skeletal survey is normal.

A. Waldenstrom's macroglobulinemia
B. Amlyoidosis
C. Myeloma
D. Monoclonal Gammopathy of Unknown Significance
E. Heavy Chain Disease

 

Immunosecretory Disorders

 

A 57 yr-old woman presents with headache & dizziness; numbness & blanching of her fingers. The IEP is at right. The PB smear shows 4+ rouleaux. You find enlarged axillary & inguinal lymph nodes & splenomegaly. X-rays show no lytic bone lesions.

In comparing the precipitin arcs of the Pt with the Control you see an abnormal arc in the Pt-antiPV and a matching arc on the IgM and k antisera.

The abn-Ig has a skewed or asymetrical arc and is in excess compared to normal. Here the Pt has an IgM in excess, but little IgG, or IgA. This is interpreted as a k monoclonal IgM, but should be confirmed with immunofixation as shown NEXT.

 

HEMATOPATHOLOGY

 

The immunofixation of this patient is shown at right. Does this confirm your interpretation of the IEP?

Based on the history and these results which of the following is the best diagnosis?

A. Waldenstrom's macroglobulinemia
B. Amlyoidosis
C. Myeloma
D. Monoclonal Gammopathy of Unknown Significance
E. Heavy Chain Disease

 

 

Multiple myeloma is generally characterized by all of the following EXCEPT:

A. Monoclonal gammopathy
B. Rouleaux formation
C. Anemia
D. Splenomegaly
E. Hypercalcemia

 

In multiple myeloma the immunoglobulin most commonly elevated is:

A. IgA
B. IgG
C. IgM
D. IgD
E. IgE

 

Multiple myeloma is often associated with:

A. Decreased ESR (erythrocyte sedimentation rate)
B. Excretion of both kappa & lambda light chains in the urine
C. Osteoblastic skeletal lesions
D. Renal excretory impairment
E. Hyperalbuminemia

 

Which feature is more characteristic of Waldenstrom's macroglobulinemia than multiple myeloma?

A. Discrete lytic bone lesions
B. Bence-Jones protein in the urine
C. Rapid clinical course
D. Spleen and lymph node enlargement
E. IgA M-spike

 

Hyperviscosity is a complication of which of the following diseases:

A. Waldenstrom's macroglobulinemia
B. Sickle cell anemia
C. IgA Myeloma
D. Polycythemia vera
E. All of the above

 

All of the following are complications associated with multiple myeloma EXCEPT:
A. Pathologic fractures
B. Increased susceptibility to infections
D. Hypergammaglobulinemia
E. Cryoglobulinemia

 

Characteristic features of various heavy chain diseases include all of the following EXCEPT:
A. Increased levels of IgG kappa or lambda light chains
B. Association with AIHA, Sjogren's syndrome, SLE, etc.
C. Anemia, fever, lymphadenopathy, splenomegaly
D. abdominal mass; malabsorption; diarrhea
E. Mediterranean lymphoma

 

All of the following features of B cell gene rearrangements are true EXCEPT:
A. Two attempts at k rearrange ment must take place before an attempt at l rearrangement can take place
B. The Cm gene is physically closest to the JH region on chromosome 14
C. A heavy chain class switch allows identical VH/DH/JH antigen specificity to be expressed with different constant regions
D. Rearrangementtranslation transcription
E. The gene for k light chains is located on chromosome 2 and has a single constant region

 

All of the following are true EXCEPT:
A. Maternal IgG can cross the placenta
B. Waldenstrom's macroglobulinemia is associated with mature B lymphocytes that have not yet undergone a heavy chain class switch
C. IgA is a major immunoglobulin in GI tract and respiratory secretions
D. The Fab portion of IgG is important in the activation of complement
E. a2-macroglobulin is a protease inhibitor

 

Navigation Bar