J Rheum Dis.  2011 Dec;18(4):297-301. 10.4078/jrd.2011.18.4.297.

A Case of Type I Cryoglobulinemia Associated with Smoldering Myeloma and Acutely Reactivated Chronic Hepatitis B

• Kim HJ
• Hong YH
• Lee HS
• Kim MJ

• KMID: 2223134
• DOI: http://doi.org/10.4078/jrd.2011.18.4.297

Abstract

A 58-year-old male patient with chronic hepatitis B infection and hypertension was referred for the evaluation of a skin rash. The skin biopsy showed multiple hyaline thrombi in small blood vessels, red blood cell extravasation, and epidermal atrophy. The CBC, chemistry, UA, and radiological studies were unremarkable except for elevated AST/ALT on liver function tests. The hepatitis B virus markers were compatible with the diagnosis of acute replicative phase chronic hepatitis B; HBeAg 86,646 cpm (count per minute), anti-HBeAb (-), HBV PCR (Quantitative) >1.10x108 IU/mL, and >640,200,000 copies/mL. Rouleaux formation was seen on the peripheral blood smears. Serum PEP/IEP demonstrated an M-spike (27.53%) in the gamma region and abnormal bowed arcs in IgG, kappa light chain with Cryoglobulin (+), ANCA (+), FANA (-), and rheumatoid factor (-) on the serological test. The percent of plasma cells on the bone marrow biopsy was approximately 15%. Type I cryoglobulinemia is a rare disease that can be associated with hematologic disorders, but smoldering myeloma or/and hepatitis B has not been reported in association with Type I cryoglobulinemia. Here, we report a case of Type I cryoglobulinemia that showed multiple skin ulcers due to vascular occlusion related to the monoclonal cryoglobulin with smoldering myeloma and acutely reactivated chronic hepatitis B.

Keyword

Cryoglobulinemia; Chronic hepatitis B; Smoldering myeloma

MeSH Terms

Antibodies, Antineutrophil Cytoplasmic
Atrophy
Biopsy
Blood Vessels
Bone Marrow
Cryoglobulinemia
Erythrocytes
Exanthema
Hepatitis B
Hepatitis B e Antigens
Hepatitis B virus
Hepatitis B, Chronic
Hepatitis, Chronic
Humans
Hyalin
Hypertension
Immunoglobulin G
Light
Liver Function Tests
Male
Middle Aged
Plasma Cells
Polymerase Chain Reaction
Rare Diseases
Rheumatoid Factor
Serologic Tests
Skin
Skin Ulcer
Antibodies, Antineutrophil Cytoplasmic
Hepatitis B e Antigens
Immunoglobulin G
Rheumatoid Factor

Figure

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  Figure 1. Multiple rice sized ery-themas and purpuric papules were seen on the both lower extremities.

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  Figure 2. Skin biopsy shows (A) hyaline thrombi in venules (×400, H & E stain), and (B) extravasation of RBC and epidermal atrophy (×200, H & E stain).

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  Figure 3. (A) A protein electro-phoresis shows an M-spike. (B) Serum IEP pattern shows moderate increase of gamma globulin area in polyvalent antiserum, and abnormal bowed arcs in IgG, kappa light chain monovalent antisera.

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  Figure 4. Bone marrow biopsy shows a mixture of myeloid cells, erythroid cells, hematopoietic cell, and increased plasma cells (A; ×400, H & E satin, B; ×400, VS3-8C stain for plasma cell).

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  Figure 5. After 2 months of treatment, even though the skin pigme-ntation remained there developed no new lesion.

Reference

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