High-Risk Kappa Light Chain Multiple Myeloma with Cast Nephropathy: A Case Report

High-Risk Kappa Light Chain Multiple Myeloma with Cast Nephropathy: A Case Report

 This is an online E log book to discuss our patient's de-identified health data shared after taking his/her/guardian's signed informed consent. Here we discuss our individual patient's problems through series of inputs from available global online community of experts with an aim to solve those patient's clinical problems with collective current best evidence based inputs. This E log book also reflects my patient-centered online learning portfolio and your valuable inputs on the comment box

Abstract

We present a case of a 58-year-old female diagnosed with high-risk Kappa light chain Multiple Myeloma (MM) with cast nephropathy requiring hemodialysis. Notable features include del(17p) and del(13q) cytogenetic abnormalities, successful transition from CyBorD to VTd regimen, and improvement in renal function. This case demonstrates the importance of risk-adapted therapy and treatment modification based on clinical response.

Introduction

Multiple myeloma with cast nephropathy and high-risk cytogenetics represents a therapeutic challenge requiring careful balance of treatment intensity and toxicity management. The presence of del(17p) is associated with poor prognosis, necessitating optimal therapeutic strategies.

Case Presentation

Initial Presentation (Early 2023):

- 58-year-old female with hypertension presented with:

  - Easy fatiguability

  - Lower limb swelling

  - Facial puffiness

  - Weight loss and poor appetite

- Initial labs showed severe anemia (Hb 6.3) and renal dysfunction

Diagnostic Workup:

1. Initial Findings:

- High FLC ratio: 4056.5

- Beta-2 microglobulin: >20,500

- Bone marrow: 80% plasma cells

- Kidney biopsy: Cast nephropathy with kappa light chain deposits

- FISH: del(13q), del(17p) - indicating high-risk disease

- Cytogenetics: 46XX, der(2)t(1;2),(q21;q37)

Treatment Course:

Phase 1 (Early-Mid 2023):

- Started on CyBorD (Cyclophosphamide, Bortezomib, Dexamethasone)

- Required hemodialysis for AKI

- Improved enough to transition to ambulatory care

Phase 2 (Mid 2023):

- Switched to VTd (Bortezomib, Thalidomide, Dexamethasone)

- Completed 4 cycles

Phase 3 (Late 2023):

- Changed to KPD regimen

- Achieved Complete Response (CR) after 3 cycles

Phase 4 (January 2024):

- Underwent Autologous Bone Marrow Transplant

- Started pomalidomide maintenance in April 2024

Current Status (December 2024-January 2025):

1. Disease Status:

- Biochemical progression on pomalidomide

- M band present

- K/LAMBDA ratio: 6044

- LDH: 885

2. Current Labs (11.1.25):

- Hb: 8 g/dL

- TLC: 4400

- Platelets: 35k

- Creatinine: 5.5

- Urea: 134

3. Current Treatment:

- Started CPd (Cyclophosphamide + Pomalidomide + Dexamethasone) on 31.12.24

- Treatment Protocol:

  - Pomalidomide 4mg D1-21

  - Cyclophosphamide 500mg D1,D8,D15,D22

  - Dexamethasone 40mg D1,D8,D15,D22

4. Supportive Care:

- Ecosprin 75mg (anticoagulation)

- Acyclovir 200mg BD (antiviral prophylaxis)

- Salbutamol nebulization

- Regular nephrology follow-up

This case demonstrates:

1. High-risk myeloma management

2. Complex treatment sequencing

3. Management of renal complications

4. Importance of maintenance therapy

5. Handling of disease progression after transplant

6. Role of supportive care in management

The patient's journey illustrates the typical progression of high-risk multiple myeloma, showing initial response, transplant consolidation, maintenance, and eventual progression requiring subsequent lines of therapy.​​​​​​​​​​​​​​​​

At admission : ( 14-1-25 ) 

Patient is asymptomatic 

On routine evaluation - platelets 7000 cells 

1 unit SDP transfusion done 

In view of severe thrombocytopenia Cyclophosphamide and pomalidomide was withholded as per medical oncologist advice. 

Certainly, let's analyze the blood smear step by step.

Overall Impression:

The blood smear shows several abnormalities suggesting a complex hematological picture, likely related to the patient's history of multiple myeloma and post-bone marrow transplant status.

Specific Findings:

 * WBC count: 3.6 x 10^9/µL - This is within the normal range.

 * Differential:

   * Neutrophils (NE): 45.7% - Normal range.

   * Lymphocytes (LY): 30.2% - Normal range.

   * Monocytes (MO): 20.9% - Elevated, suggesting a possible reactive process or an underlying inflammatory condition.

   * Eosinophils (EO): 2.8% - Normal range.

   * Basophils (BA): 0.4% - Normal range.

 * RBC count: 3.84 x 10^12/µL - Low, indicating anemia.

 * Hemoglobin (HGB): 9.6 g/dL - Low, confirming anemia.

 * Hematocrit (HCT): 32.7% - Low, consistent with anemia.

 * Mean Corpuscular Volume (MCV): 85.2 fL - Normal, suggesting normocytic anemia.

 * Mean Corpuscular Hemoglobin (MCH): 25.0 pg - Normal.

 * Mean Corpuscular Hemoglobin Concentration (MCHC): 29.4 g/dL - Normal.

 * Red Cell Distribution Width (RDW-CV): 15.6% - Elevated, suggesting anisocytosis (variation in red blood cell size).

 * Red Cell Distribution Width (RDW-SD): 53.2 fL - Elevated, further supporting anisocytosis.

 * Platelet count (PLT): 53 x 10^9/µL - Low, indicating thrombocytopenia.

 * Plateletcrit (PCT): 0.04% - Low, consistent with thrombocytopenia.

 * Mean Platelet Volume (MPV): 7.4 fL - Normal.

 * Platelet Distribution Width (PDW): 19.2% - Elevated.

Interpretation:

 * Anemia: The low RBC count, HGB, and HCT confirm the presence of anemia. The normocytic MCV suggests that the anemia is not due to a deficiency in cell size. The elevated RDW indicates significant variation in red blood cell size and shape, which can be seen in various conditions, including anemia of chronic disease, iron deficiency, and myelodysplastic syndromes.

 * Thrombocytopenia: The low platelet count is a significant finding. It can be due to various causes, including bone marrow suppression, increased platelet destruction, or consumption. Given the patient's history of multiple myeloma and bone marrow transplant, bone marrow suppression is a likely possibility.

 * Monocytosis: The elevated monocyte count could be a reactive response to an underlying infection or inflammation. It could also be related to the patient's underlying malignancy or the effects of the bone marrow transplant.

Admission 2 : 

Patient presented with chief  complaints of dark coloured patches on forearm since -4 days and worsening cough and SOB since a week. 

soap update date 2 : 

[23/01/25, 2:03:47 PM] Sai Charam Kulkarni: Update on this case : 

S- patient able to sleep on bed, cough and sob decreased No fresh echymotic patches 

O - bilateral crepts present RR - 26 cpm difficulty in completing sentences Orthopnea decreased slightly She is better in sitting than lying down Peripheral Smear shows plasma cells ( will share images shortly ) 

HRCT chest video : https://youtube.com/shorts/IDyLw0kMHeI?feature=share

A - relapse of Multiple myeloma S/P Bone marrow transplant Cast nephropathy Left upper lobe pneumonia Rt Fungal ball

 P - Anti fungal added 

Questions around this case : Can it be ITP in MM triggered by fungal pneumonia ..? She doesn’t have any bleeding manifestations when her platelets was 7000 but now she have this patches at platelets 16000..?

[23/01/25, 2:38:16 PM] Dr R B: Thanks for sharing. Really heartening to see your progress!  Well your patient is currently on cyclophosphamide and pomalidomide, and a significant side effect that can occur with this combination is thrombocytopenia, this is considered one of the most common grade 3 or 4 hematological adverse events associated with this combination therapy, often requiring close monitoring during treatment. https://pmc.ncbi.nlm.nih.gov/articles/PMC7983889/#:~:text=Pomalidomide%20was%20temporarily%20interrupted%20for%20patients%20with,other%20grade%20%E2%89%A5%203%20treatment%E2%80%90related%20adverse%20events.&text=The%20most%20common%20grade%203%20or%204,neutropenia%20(20.0%)%2C%20anemia%20(10.9%)%2C%20and%20thrombocytopenia%20(18.2%). ‎

[23/01/25, 4:20:38 PM] Dr R B: Abdominal fat pad has a poor sensitivity! Can't rule out amyloid if negative  https://www.sciencedirect.com/science/article/abs/pii/S2213294524000036

[23/01/25, 4:07:46 PM] Sai Charam Kulkarni: Bullous hemorrhagic lesions are more common ( > 50% ) in AL myeloma than echymosis or purpura.

[23/01/25, 2:54:16 PM] Sai Charam Kulkarni: The treatment‐emergent adverse events (TEAEs) are shown in Table 3. The most common grade 3 or 4 hematological adverse events were neutropenia (20.0%), anemia (10.9%), and thrombocytopenia (18.2%). The most common grade 3 or more non‐hematological adverse events were pneumonia (21.8%), febrile neutropenia (14.6%) and sepsis (9.0%). The seven deaths recorded during the study period were due to pneumonia (four cases), sepsis (two cases), and cardiac arrest (one case) From same case report sir